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1.
Opinion statement  
–  Drug-induced liver disease can result from dosage-dependent hepatotoxicity or from adverse reactions to drugs used in therapeutic dosage. The latter idiosyncratic hepatotoxins can cause clinical syndromes that mimic all known liver diseases, so that drugs must be considered as the possible causal agent for all unexplained cases of liver disease.
–  The only specific antidote for dosage-dependent hepatotoxicity is N-acetylcysteine (and some other sulfhydryl donors), which is highly effective for the prevention of significant hepatotoxicity after acetaminophen overdose.
–  Early diagnosis and prompt withdrawal of the offending drug is the key to successful management of most drug-induced liver diseases.
–  The mainstay of treatment is supportive care, with careful monitoring for signs of acute liver failure or progression to chronic liver disease.
–  In cases of liver failure, close liaison with a liver transplant center is crucial; referral for liver transplantation should be considered if standard transplant criteria are fulfilled.
–  Pruritus is a major symptom of drug-induced cholestasis; protracted cases may respond to ursodeoxycholic acid.
–  Corticosteroids can be considered for cases of drug-induced hepatitis, especially those with evidence of immune hypersensitivity, if no improvement is seen in 8 to 12 weeks. Although there are no controlled trials, some patients may respond favorably.
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2.
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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3.
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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4.
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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5.
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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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  
–  In the United States, the majority of patients with granulomatous liver disease do not have an identifiable cause.
–  When a specific diagnosis can be made, therapy should be directed at eradicating the cause.
–  A plan of management must recognize the drug history and nationality of the patient, chest radiograph, slit lamp examination of the eyes, skin tests, special stains of liver biopsies, and serum antibodies.
–  In those patients for whom a specific diagnosis cannot be established, the use of corticosteroids requires that the patient have a clinical justification for treatment or manifest important changes in findings of liver biopsies.
–  Patients selected for steroid treatment should be subjected to close laboratory monitoring, and the decision to continue therapy should be based on objective parameters.
–  Treatment of steroid-resistant disease might require methotrexate, chloroquine, cyclosporine, azathioprine, or ursodeoxycholic acid.
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8.
Amebiasis     
Opinion statement  
–  More than 80% of cases of amebic liver abscess can be managed with a 14-day course of intravenous or oral metronidazole. In cases of suspected amebic liver abscess, treatment should be started before diagnostic confirmation.
–  If no clinical improvement is evident by 72 to 96 hours, treatment should be changed to dehydroemetine and chloroquine.
–  Invasive treatment is necessary only in patients in whom medical treatment fails within 5 days or in whom signs of clinically severe disease are present.
–  A 10-day course with a luminal agent such as paromomycin to eliminate intestinal cysts, which are resistant to imidazoles, should always follow treatment of the liver abscess.
–  Percutaneous catheter drainage is indicated in patients with impending rupture, with a lesion 6 cm or more in diameter, with an abscess located in the left lobe or high in the dome of the right lobe, or in whom medical treatment fails.
–  Although sympathetic pleural effusion is not an indication for drainage, direct pulmonary involvement or spread to pleural or lung tissues requires drainage.
–  Intraperitoneal rupture and peritonitis necessitate open surgical drainage.
–  Only a small minority of amebic liver abscesses are secondarily infected by other organisms.
–  Because relapses are possible, feces should be checked for cysts monthly for several months after therapy.
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9.
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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10.
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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11.
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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12.
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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13.
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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14.
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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15.
Hepatitis B     
Opinion statement  
–  The management of acute HBV infection is supportive.
–  Specific treatment is not indicated for HBV carriers because they often have no evidence of liver injury, and, further, do not respond to currently available therapies.
–  Interferon monotherapy is best indicated for patients with chronic replicating HBV infection and evidence of chronic hepatitis. There is an increased likelihood of clearing HBsAg with interferon monotherapy as compared to lamivudine.
–  Lamivudine is an oral nucleoside analog that is better tolerated than interferon. The clinical situations for its use are far more than interferon monotherapy. Lamivudine should be used in patients with decompensated cirrhosis and also in transplantation, both before and after transplantation.
–  The post-transplant use of hepatitis B immune globulin (HBIG) and lamivudine combination therapy may be better for recipients who are identified in a replicative phase prior to transplantation.
–  Hepatitis B coinfection with one or more viruses, HCV, HDV, or HIV, may occur. Both interferon and lamivudine have been useful in these patients. However, the data are sparse and heterogeneous. Therapy with one or both drugs will have to be tailored to the clinical situation.
–  Combination therapy with immunomodulatory and/or antiviral drugs are what we will be looking toward in the future.
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16.
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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17.
Choledocholithiasis in the Setting of Portal Hypertension   总被引:2,自引:0,他引:2  
Opinion statement  
–  The successful management of biliary lithiasis in patients with cirrhosis depends on the degree of liver dysfunction. Patients with Child-Pugh class A or B hepatic reserve have excellent prognoses.
–  Endoscopic spincterotomy is first-choice treatment for cirrhotic patients with choledocholithiasis. Endoscopic sphincterotomy can be performed in patients with Child-Pugh class A, B, or C hepatic function.
–  Medical treatment with contact solvents is contraindicated in patients with pigment stones or calcified stones.
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18.
Opinion statement  
–  Most symptomatic internal hemorrhoids, grade 1 through 3, can be treated successfully with office-based procedures.
–  Anorectal suppurative diseases must be treated surgically. Control of sepsis with subsequent fistula surgery as necessary is the goal.
–  New nonoperative methods of anal fissure therapy are directed at reducing anal sphincter pressures. These methods have shown significant reduction in the need for sphincterotomy—a proven surgical technique with some risk of impaired continence.
–  Surgery, using an advancement flap and partial internal sphincterotomy, remains the primary treatment for anal stenosis.
–  Solitary rectal ulcer remains a difficult problem to manage medically and surgically.
–  Multiple surgical techniques can effectively treat rectal prolapse. A minimal technique using Silastic wrap (Wright Medical Technologies; Arlington, TX), perineal resection (Altemeier procedure), and sigmoidectomy-rectopexy, or Ripstein suspension, has been the most favored method in selected patients.
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19.
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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20.
Hepatitis C     
Opinion statement  
–  End-stage liver disease due to chronic hepatitis C is the leading indication for orthotopic liver transplantation in the United States. Twenty percent to 30% of hepatitis C patients are at increased risk of developing cirrhosis, and 1% to 4% of cirrhotic patients will develop hepatocellular carcinoma. These findings warrant treatment for hepatitis C virus (HCV)-infected patients.
–  Currently, the mainstay in treatment of HCV is the use of recombinant alpha interferon, or its equivalent, in combination with the oral antiviral agent ribavirin.
–  The major goals of therapy are clearance of the virus, achieving a noninfectious state, and halting the necro-inflammatory process that leads to fibrosis and progression to cirrhosis.
–  End of treatment response (ETR) is biochemical and virological remission—normalization of serum aminotransferase (ALT) and undetectable levels of HCV RNA, at the end of therapy.
–  Sustained virological response (SVR) is defined as the absence of viremia and persistently normal aminotransferase 6 months off treatment, and is the ultimate goal of therapy. Patients who achieve SVR will have significant and persistent histologic improvement.
–  HCV genotype, pretreatment levels of HCV-RNA (viral load), the presence of advanced fibrosis or cirrhosis, gender, and age are independent predictors of response.
–  Ribavirin is teratogenic, therefore, contraception is mandatory for both males and females during and up to 6 months after therapy.
–  Side effects of combination therapy are dose-dependent and most commonly include symptoms of irritability, depression and fatigue, and laboratory evidences of leukopenia, thrombocytopenia, and hemolytic anemia.
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