e1152 e1 Viral Hepatitis (A, B, C, D, E), CMV, HSV, EBV, VZV, HHV 6, parvovirus B19, parainfluenza, yellow fever Idiosyncratic Halogenated hydrocarbons, warfarin, methyldopa, phenytoin, carbamazepine,[.]
e1152.e1 eTABLE Diseases That Cause Liver Failure 95.2 Viral Hepatitis (A, B, C, D, E), CMV, HSV, EBV, VZV, HHV-6, parvovirus B19, parainfluenza, yellow fever Idiosyncratic Halogenated hydrocarbons, warfarin, methyldopa, phenytoin, carbamazepine, valproic acid, rifampicin, penicillin, sulfonamides, quinolones Toxic, dose dependent Acetaminophen (paracetamol), isoniazid, tetracycline, methotrexate, carbon tetrachloride, amphetamines, Amanita phalloides toxin Toxic, synergistic Ethanol acetaminophen, barbiturate acetaminophen, isoniazid rifampicin Metabolic Wilson disease, alpha-1-AT deficiency, galactosemia, tyrosinemia, Reye syndrome, NASH Associated with pregnancy Acute fatty liver of pregnancy, HELLP syndrome Vascular Budd-Chiari syndrome, veno-occlusive disease, shock, heart failure, Fontan procedure Miscellaneous Autoimmune hepatitis, malignant infiltration, hyperthermia, sepsis CMV, Cytomegalovirus; EBV, Epstein-Barr virus; HELLP, hemolysis, elevated liver enzymes, and low platelet count; HHV, human herpesvirus; HSV, herpes simplex virus; NASH, nonalcoholic steatohepatitis; VZV, varicella zoster virus Data from Gotthardt D, et al Fulminant hepatic failure: etiology and indications for liver transplantation Nephrol Dialysis Transplant 2007;22(suppl 8):viii5–viii8 CHAPTER 95 Disorders and Diseases of the Gastrointestinal System pressure decreases or portal pressure increases Patients who are hyponatremic usually have total-body sodium overload.95 They should be treated with moderate salt restriction; however, vigorous fluid restriction, colloid administration, and diuretic therapy are the cornerstones of therapy High-volume abdominal paracentesis is proved to be safe in both adults and children who have ascites compromising ventilation Spontaneous engorgement of esophageal, gastric, and rectal veins leads to varices Similar prominent veins in the abdominal wall and around the umbilicus (caput medusa) may develop Splenic congestion from impaired venous flow into the portal system results in splenomegaly and hypersplenism so that patients are classically anemic or pancytopenic Less commonly, unknown factors lead to intrapulmonary arteriovenous shunting, which characteristically causes hepatopulmonary syndrome or portopulmonary hypertension and subsequent hypoxemia Investigators have recently become aware of a high risk for cardiomyopathy in cirrhotic patients Because of increased sympathetic tone and a hyperdynamic circulatory state, cardiac muscle may become remodeled, resulting in hypertrophy and heart failure unmasked by physiologic stress.97 Coagulopathy and bleeding are frequent in patients with liver failure Thrombocytopenia, which may be profound, is a common component of hypersplenism Fat-soluble vitamin malabsorption in patients with cholestasis leads to vitamin K deficiency, which prevents hepatic production of clotting factors II, VII, IX, and X Ultimately, failed synthesis of all liver-dependent clotting factors results in prolonged prothrombin time (unresponsive to vitamin K administration) and partial thromboplastin time The sites of bleeding are predictable Bleeding from incisions, needle puncture sites, the nose, and gingiva are common but are usually not life threatening Persistent bleeding may require packed red blood cell transfusion In contrast, intracranial, pulmonary, and variceal bleeding may be fatal and demand immediate attention Esophageal varices bleed because of acute changes in variceal pressure or because of gastric hyperacidity It is notable that blood cultures are frequently positive during acute variceal hemorrhage; some hepatologists advocate for empiric use of broad-spectrum antibiotics when bleeding varices are encountered.98 Platelets should be given to patients with thrombocytopenia and active bleeding or prior to a procedure Patients with coagulopathy and hemorrhage may receive fresh frozen plasma, but if not actively bleeding, it is best to allow the international normalized ratio (INR) to rise, as continual replacement of blood product can lead to fluid overload in these already sensitive patients Acceptable alternatives include plasmapheresis99 and the administration of recombinant factor VII concentrate100 as bridges to transplantation (see also Chapter 89) When factor VII concentrate is used, care should be taken to actively modify the dose to prevent clot development While correcting severe coagulopathy during active bleeding may be helpful, it is also important to note that current practice is to refrain from overcorrection of thrombopathy insofar as most patients in liver failure function in a tight balance between depletion of clotting factors and depletion of thrombophilic factors such as protein C, protein S, and antithrombin Thus, many patients with elevated INRs or low platelets are not at risk for bleeding because coagulation and fibrinolysis remain within an acceptable balance.101 Mechanical means of hemorrhage control include direct compression, creation of portosystemic shunts, and surgical transection/reanastomosis of the esophagus Endoscopic control of 1153 bleeding from varices involves sclerotherapy in infants too small to tolerate a banding device or endoscopic banding in toddlers and older children.102 Balloon tamponade by a Sengstaken-Blakemore tube carries substantial risk and has largely been abandoned It should not be used to manage persistent bleeding after sclerotherapy because of the risk of esophageal perforation Complications of portal hypertension can be managed with surgical shunting procedures that decompress the portal system by creating a venous anastomosis between the portal and systemic circulations Several surgical varieties exist Central vascular shunts, such as portocaval or mesocaval shunts, carry substantial risk Transjugular intrahepatic portosystemic shunts are frequently used in children who weigh more than 10 kg as a bridge to transplantation.103 In the past, shunt thrombosis was sometimes treated with surgical esophageal transaction, but urgent liver transplantation with either a cadaveric or living related donor is the preferred therapeutic strategy under such conditions (see Chapter 97) Plasmapheresis or plasma exchange removes circulating mediators and toxins from patients with liver failure This temporary effect may be due to removal of neuroinhibitory factors The molecular adsorbent recirculating system and other similar albumin dialysis systems, which remove large, albumin-bound molecules such as bilirubin and ammonia, have now been used for several years as a bridge to liver transplantation These systems have shown an improvement in hepatic encephalopathy and bilirubin with a variable impact on mortality in adults; however, pediatric data remain limited.104 Ultimately, orthotopic transplantation holds the greatest promise for patients with end-stage liver disease.105 Key References Anderson KD, Rouse TM, Randolph JG A controlled trial of corticosteroids in children with corrosive injury of the esophagus N Engl J Med 1990;323:637-640 Ardura M, Lewis J, Tansmore JL, Harp PL, Dienhart MC, Balint JP Central catheter-associated bloodstream infection reduction with ethanol lock prophylaxis in pediatric intestinal failure JAMA Pediatr 2015;169:324-331 Cohen EB, Afdhal NH Ultrasound-based hepatic elastography; origins, limitations, and applications J Clin Gastroenterol 2010;44:637-645 Cook DJ, Reeve BK, Guyatt GH, et al Stress ulcer prophylaxis in critically ill patients Resolving discordant meta-analyses JAMA 1996;275:308-314 Dranove JE Focus on diagnosis: new technologies for the diagnosis of gastroesophageal reflux disease Pediatr Rev 2008;29:317-320 Hong SK, Vaezi MF Gastroesophageal reflux monitoring: pH (catheter and capsule) and impedance Gastrointest Endosc Clin North Am 2009;19:1-22 Kalach N, Bontems P, Cadranel S Advances in the treatment of Helicobacter pylori infection in children Ann Gastroenterol 2015;28:10-18 Karabiber H, Selimoglu MA, Otlu B, Yildirim O, Ozer A Virulence factors and antibiotics resistance in children with Helicobacter pylori gastritis J Pediatr Gastroenterol Nutr 2014;58:608-612 Mezoff EA, et al Ethanol lock efficacy and associated complications in children with intestinal failure JPEN J Parenter Enteral Nutr 2016; 40:815–819 Millar AJ, Cox SG Caustic injury of the oesophagus Pediatr Surg Int 2015;31:111-121 Moazam F, Talbert JL, Miller D, Mollitt DL Caustic ingestion and its sequelae in children South Med J 1987;80:187-190 Montgomery J, McCusker S, Hendry J, Lumley E, Kubba H Botulinum toxin A for children with salivary control problems Int J Pediatr Otorhinolaryngol 2014;78:1970-1973 1154 S E C T I O N X Pediatric Critical Care: Gastroenterology and Nutrition Noonan K, Prunty S, Ha JF, Vijayasekaran S Surgical management of chronic salivary aspiration Int J Pediatr Otorhinolaryngol 2014;78: 2079-2082 Partovi S, Kohan A, Rubbert C, et al Clinical oncologic applications of PET/MRI: a new horizon Am J Nucl Med Mol Imaging 2014;4: 202-212 Robinson J, Casey LM, Huynh HQ, Spady DW Prospective cohort study of the outcome of and risk factors for intravascular catheter-related bloodstream infections in children with intestinal failure JPEN J Parenter Enteral Nutr 2014;38:625-630 Van Beers B, Daire JL, Garteiser P New imaging techniques for liver disease J Hepatol 2015;62:690-700 The full reference list for this chapter is available at ExpertConsult.com e1 References Figueroa-Colon R, Grunow JE Randomized study of premedication for esophagogastroduodenoscopy in children and adolescents J Pediatr Gastroenterol Nutr 1988;7:359-366 Costamagna G, Shah SK, Riccioni ME, et al A prospective trial comparing small bowel radiographs and video capsule endoscopy for suspected small bowel disease Gastroenterology 2002;123:999-1005 Liangpunsakul S, Chadalawada V, Rex DK, et al Wireless capsule endoscopy detects small bowel ulcers in patients with normal results from state of the art enteroclysis Am J Gastroenterol 2003;98: 1295-1298 de Leusse A, Vahedi K, Edery J, et al Capsule endoscopy or push enteroscopy for first-line exploration of obscure gastrointestinal bleeding? 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Ardura M, Lewis J, Tansmore JL, Harp PL, Dienhart MC, Balint JP Central catheter-associated bloodstream infection reduction with ethanol lock prophylaxis in pediatric intestinal failure JAMA Pediatr 2015;169:324-331 25 Robinson J, Casey LM, Huynh HQ, Spady DW Prospective cohort study of the outcome of and risk factors for intravascular catheterrelated bloodstream infections in children with intestinal failure JPEN J Parenter Enteral Nutr 2014;38:625-630 26 Percival SL, Kite P, Eastwood K, et al Tetrasodium EDTA as a novel central venous catheter lock solution against biofilm Infect Control Hosp Epidemiol 2005;26:515-519 27 Percival SL, Salisbury AM The efficacy of tetrasodium EDTA on biofilms Adv Exp Med Biol 2018;1057:101-110 28 Liu F, Hansra S, Crockford G, et al Tetrasodium EDTA Is Effective at Eradicating Biofilms Formed by Clinically Relevant Microorganisms from Patients’ Central Venous Catheters mSphere 2018;3(6):e00525-18 29 Moazam F, Talbert JL, Miller D, Mollitt DL Caustic 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INRs or low platelets are not at risk for bleeding because coagulation and fibrinolysis remain within an acceptable balance.101 Mechanical means of hemorrhage control include direct compression,... Plasmapheresis or plasma exchange removes circulating mediators and toxins from patients with liver failure This temporary effect may be due to removal of neuroinhibitory factors The molecular adsorbent recirculating... New imaging techniques for liver disease J Hepatol 2015;62:690-700 The full reference list for this chapter is available at ExpertConsult.com e1 References Figueroa-Colon R, Grunow JE Randomized