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1.
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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2.
Opinion statement  
–  The key to successful management of biliary tract carcinoma is early diagnosis and a multidisciplinary team approach involving a hepatobiliary surgeon, therapeutic endoscopist, and oncologist.
–  Management depends on the location of the tumor ( proximal or distal), and resectability.
–  Distal tumors that are resectable are best treated with radical pancreaticoduodenectomy, while nonresectable distal tumors are treated by using endoscopic or percutaneous biliary stents for biliary decompression.
–  Treatment of proximal resectable tumors depends on the Bismuth Classification. Bismuth I and II tumors can be resected without concomitant hepatic resection, and Bismuth III and IV tumors require local tumor and hepatic parenchymal resection. Nonresectable proximal tumors are best treated with percutaneous transhepatic biliary stents.
–  The role of chemotherapy, radiotherapy, and photodynamic therapy in these patients remains investigational.
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3.
Gastric Cancer     
Opinion statement  
–  The best treatment for gastric cancer remains early detection and prompt surgical removal. Currently, the majority of gastric carcinomas diagnosed in western centers are late-stage tumors with poor prognoses.
–  Endoscopic ultrasound and diagnostic laparoscopy aid our ability to accurately stage patients with gastric cancers, and may prevent unnecessary laparotomies in asymptomatic patients.
–  While extended lymphadenectomy is the standard approach in Japanese centers, the utility of this procedure in improving long-term survival has yet to be proved in prospective, randomized trials.
–  Accurate staging of patients preoperatively and selected multidisciplinary approaches to individual patients may eventually lead to improvements in survival for patients with gastric cancer.
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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  
–  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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6.
Opinion statement  
–  Tumors of the liver often are discovered incidentally in asymptomatic individuals during diagnostic imaging or exploratory laparotomy performed for alternative reasons.
–  Hemangiomas are the most common benign liver tumors, followed in prevalence by focal nodular hyperplasia (FNH); other benign tumors are rare.
–  The growth and development of hemangiomas, FNH, and hepatic adenomas especially, have been linked to hormonal (eg, estrogen) stimulation.
–  Differentiating between benign and malignant neoplasms of the liver can be challenging. Radiologic imaging is essential for preliminary identification and classification of hepatic tumors, but tissue biopsy or surgical excision sometimes is required for a definitive diagnosis.
–  Individuals with hemangioma or FNH usually are asymptomatic, have a benign course, and can be managed conservatively. In contrast, hepatic adenomas need to be followed more carefully and often are excised to provide symptomatic relief, remove the risk of rupture, and avoid potential malignant transformation.
–  Liver transplantation in patients with benign hepatic neoplasms is exceedingly uncommon, and is recommended only when alternative methods of treatment are not feasible or have failed to control significant symptoms.
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7.
Opinion statement  
–  Balloon angioplasty or early surgical correction is recommended once coarctation of the aorta has been diagnosed. Medical management is not a long-term option.
–  The rate of restenosis is higher in infants and children than in adults, but the incidence of residual or late hypertension increases with age at initial intervention.
–  Current angioplasty and stent placement techniques have reduced the rate of aneurysm formation and expanded the types of complex anatomic configurations amenable to nonsurgical intervention.
–  Patients require long-term follow-up for restenosis and late or residual hypertension.
–  Coarctation treatment is straightforward in patients with simple isolated coarctation. In neonates with associated lesions and diffuse arch hypoplasia, aggressive (albeit higher-risk) arch-enlargement procedures can be done at the time of open-heart correction of intracardiac defects.
–  Changes in aortic compliance, vascular reactivity, and vascular homeostasis mechanisms in patients who have late surgery are associated with a higher incidence of residual hypertension.
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8.
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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9.
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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10.
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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11.
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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12.
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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13.
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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14.
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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15.
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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16.
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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17.
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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18.
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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19.
Opinion statement  
–  Lack of endoscopic esophagitis does not exclude gastroesophageal reflux disease (GERD).
–  Ambulatory pH testing is also an imperfect standard, and patients with both a normal endoscopy and a normal pH test may still have symptoms produced by acid reflux.
–  A therapeutic trial of acid suppression is often the best approach to these patients.
–  Ideally, therapeutic trials should use a medication with a high degree of efficacy in the treatment of GERD to avoid a false-negative test.
–  Proton pump inhibitors (PPIs) are the best currently available medical therapy for all forms of GERD.
–  If the patient does not respond to a once daily PPI, options include increasing the dose of PPIs, and, perhaps, adding another class of agent or studying the patient with an ambulatory pH test.
–  Patients with a negative endoscopy, negative pH test. and those who do not respond to an adequate trial of acid suppression are unlikely to benefit from antireflux surgery.
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20.
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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