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1.
Opinion statement  
–  Angiographic restenosis occurs in 30% to 50% of patients after percutaneous transluminal coronary angioplasty (PTCA) with 20% to 30% target vessel revascularization at one year, and is associated with increased morbidity, mortality and health care costs.
–  Intracoronary stents are the first line of therapy against restenosis after angioplasty. Depending on lesion morphology and location, stents can reduce restenosis and target lesion revascularization (TLR) by 20% to 30%.
–  Obstructive coronary lesions in vessels with a diameter larger than 3.0 mm should be stented. The benefit of stenting in vessels smaller than 3.0 mm is controversial, with the BESMART (Bestent in Small Arteries) and ISAR-SMART (Intracoronary Stenting or Angioplasty for Restenosis Reduction in Small Arteries) studies demonstrating conflicting results. Chronically occluded and subtotal vessels should be stented after PTCA. Obstructive lesions in saphenous vein grafts should be stented. It is preferable to stent ostial lesions after PTCA.
–  Restenosis can occur in 15% to 25% of patients within 6 months of stent placement. Initial approach to focal in-stent restenosis is to repeat PTCA. Patients with diffuse restenosis may require debulking prior to PTCA to improve acute results. "Stenting within-stent" has not proven beneficial unless there is diffuse in-stent restenosis, neointimal prolapse or vessel dissection during PTCA.
–  There are no pharmacologic therapies approved by the Food and Drug Administration available to treat restenosis at present.
–  Brachytherapy, gamma or beta, is an effective adjunctive therapy that can reduce recurrent in-stent restenosis by 40% to 70%. Patients at high risk for recurrent in-stent restenosis (proliferative or total occlusion pattern) can be considered for brachytherapy to treat the first episode of in-stent restenosis. Patients with focal in-stent restenosis should be treated with brachytherapy after multiple recurrences of in-stent restenosis.
–  Emerging therapies for treatment of restenosis include antiproliferative-coated stents and photoangioplasty.
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2.
Opinion statement  
–  Balloon valvuloplasty provides optimal treatment for moderate and severe pulmonary valve stenosis.
–  Dysplastic pulmonary valves may not respond to balloon dilation and frequently require surgical treatment.
–  Balloon angioplasty with or without stenting is the preferred treatment of peripheral pulmonary stenosis.
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3.
Opinion statement  
–  Transposition of the great arteries (TGA) is a lethal condition without intervention.
–  Cross-sectional echocardiography is the diagnostic investigation of choice.
–  Intravenous infusion of prostaglandin is employed to maintain ductal patency and allow mixing of blood, thus improving tissue oxygenation.
–  Balloon atrial septostomy is recommended once the diagnosis is made.
–  The arterial switch is accepted as the best option for simple TGA.
–  Late follow-up includes survivors of the intra-atrial repair (Mustard and Senning operations), and the emerging cohort of survivors of the arterial switch procedure.
–  Arrhythmia, baffle stenosis, tricuspid valve dysfunction, systemic ventricular dysfunction, and sudden death may occur late during follow-up after the Mustard or Senning procedure.
–  There are less data for late follow-up after arterial switch; however, late death is rare, usually is related to reoperation, and important arrhythmias are uncommon. The long-term fate of the coronary circulation is unknown but coronary arterial obstruction has been reported.
–  Continuing long-term surveillance is essential to detect the development of late problems in all groups of survivors.
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4.
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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5.
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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6.
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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7.
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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8.
Opinion statement  
–  There are four unique liver diseases that occur only during pregnancy and resolve after delivery.
–  Several liver diseases occur more commonly during pregnancy.
–  These must be distinguished form acute or chronic liver diseases that coincidentally occur during pregnancy.
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9.
Opinion statement  
–  Most of the care of liver disease in alphα1-antitrypsin (α1-AT) deficiency involves supportive management for complications of chronic liver disease including gastrointestinal bleeding, ascites, edema, encephalopathy, coagulation disturbances, spontaneous bacterial peritonitis, and hepatorenal syndrome.
–  Some of these patients will have manifestations of cholestatic injury, including pruritus, hypercholesterolemia, and steatorrhea with fat-soluble vitamin deficiencies.
–  The major challenge for the clinician taking care of these patients is the timing of referral for liver transplantation therapy.
–  Timing of such referral is a relatively straightforward decision in α1-AT-deficient patients with progressive liver dysfunction.
–  Some patients have nonprogressive or slowly progressing liver disease even after the development of cirrhosis or portal hypertension. Timing of liver transplantation in these patients should not be based simply on the presence of cirrhosis, portal hypertension or mild liver synthetic dysfunction, but rather on the basis of a subjective judgment by the hepatologist, patient, and family that manifestations of liver disease are interfering with overall life functioning.
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10.
Opinion statement  
–  Many of the childhood functional gastrointestinal disorders are extremely common.
–  Using symptom-based diagnostic criteria for pediatric functional gastrointestinal disorders will improve patient care, enhance family satisfaction, and reduce costs.
–  Using symptom-based diagnoses, the emphasis shifts from evaluations to rule out rare diseases to family education and symptom management.
–  Well-meaning clinicians may co-create disability by failure to recognize and appropriately manage functional pediatric gastrointestinal disorders.
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11.
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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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.
Hepatic cysts     
Opinion statement  
–  Treatment of hepatic cysts should be considered only for those patients who are symptomatic.
–  For simple cysts, percutaneous aspiration invariably leads to recurrence; laparoscopic deroofing is usually curative.
–  Open deroofing (fenestration) should be reserved for cysts inaccessible by laparoscopy.
–  Percutaneous instillation of sclerosing agents (ethanol, iophendylate, minocycline) into nonbiliary and nonparasitic cysts is an alternative therapeutic option in certain cases.
–  Due to increased morbidity, hepatic resection should be reserved for polycystic liver disease, diffuse hepatic involvement, or recurrence after a deroofing procedure.
–  Patients with congenital fibropolycystic disorders (eg, congenital hepatic fibrosis) with evidence of hepatic decompensation, should be considered for liver transplantation.
–  For hepatic hydatid cysts, simple cystectomy or the PAIR (puncture, aspirate, inject, and reaspirate) technique with albendazole treatment have been shown to be equally successful.
–  In the case of alveolar echinococcosis, hepatic resection and liver transplantation are the only effective modalities for localized and extensive hepatic disease, respectively.
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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  
–  The management of patients with postoperative biliary stricture is a challenging problem that spans across several disciplines.
–  A team approach involving endoscopists, interventional radiologists, and biliary surgeons is crucial for the successful management of individuals with this complex problem.
–  Hepaticojejunostomy is the therapy of choice. Prolonged stenting, either through the percutaneous route or through the endoscopic retrograde route, is an alternative.
–  Stricture recurrence is not infrequent and requires lifelong follow-up.
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16.
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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17.
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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18.
Hemochromatosis     
Opinion Statement  
–  The mainstay of treatment for hemochromatosis is therapeutic phlebotomy. The procedure is safe, effective, and relatively cheap.
–  Deferoxamine (iron chelation) is necessary only in iron-loaded individuals who cannot undergo therapeutic phlebotomy.
–  If therapy is initiated before complications develop, it is expected that no complications will ever develop.
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19.
Gallstones     
Opinion statement  
–  Gallstone disease is a common clinical problem that poses particular difficulties for our aging and increasingly obese population.
–  Exciting new research suggests an infectious cause for most cholesterol stones.
–  The keys to managing patients with gallstone disease are recognition of the wide spectrum of clinical disease resulting from gallstones and a flexible, multidisciplinary approach to diagnosis and treatment.
–  The biliary tract has been a proving ground in the development of minimally invasive technology.
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20.
Opinion statement  
–  In patients with hepatopulmonary syndrome, supplemental oxygen and liver transplantation are the usual treatments of choice. Pharmacologic approaches have limited success in improving hypoxemia. Interventional radiology procedures may improve arterial hypoxemia in highly selected patients.
–  In patients with portopulmonary hypertension, continuous infusion with intravenous epoprostenol (prostaglandin I2) can significantly improve pulmonary hemodynamics. Outcome following liver transplantation is variable; increased cardiopulmonary mortality occurs in patients with moderate to severe pulmonary hypertension.
  相似文献   

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