Table of Contents
Patients, Families and Friends
Health Care Providers
|Specific Problems: Hepatitis and Increased Jaundice|
|Health Care Providers - Problem Oriented Clinical Guidelines|
Edited by James Eckman, M.D. and Allan Platt, PA-C
Patients with hemolytic anemia will have an elevated indirect bilirubin from the increased breakdown of heme and an elevated lactic dehydrogenase from increased breakdown of erythrocytes. The level of indirect bilirubin seldom is > 4 mg.% unless there is some problem in hepatic clearance. Elevations in direct bilirubin, AST, or alkaline phosphatase suggest liver disease or increased release from cells other than erythrocytes.
Liver problems encountered in hemoglobinopathy patients include passive congestion from sickling and right heart failure, cholelithiasis, viral or alcoholic hepatitis, hepatic fibrosis/ cirrhosis, liver infiltration, and hemosiderosis/hemachromatosis. Changes in symptoms or laboratory values require evaluation.
Present Illness: Note onset of symptoms, RUQ pain, increased jaundice, dark urine, lighter stools, fever/chills, anorexia, nausea, vomiting, malaise, weight loss, oritching. Seek prodrome of arthralgias, sore throat, rash, anorexia, or malaise. Document exposure to others with hepatitis.
Past Medical History: Hospitalizations or surgery for gallstones, tumor, or drug overdose. History of transfusions including total units and last administered. Present and recent medications use especially estrogens, INH, phenothiazines, halothane, or acetaminophen. Use of alcohol and I.V. drugs. Sexual exposure.
Review of Symptoms: General ROS.
Objective DataPhysical Examination
General. Skin rash, excoriations, jaundice, cachexia, ecchymosis, palmar erythemia, spider nevi, gynecomastia.
Vital Signs. Temperature, supine and upright blood pressure/pulse.
HEENT. Scleral icterus, palor, parotid enlargement, or pharyngitis.
Neck. Posterior cervical or diffuse adenopathy.
Chest. Basilar rales, or effusions.
Heart. Venous pressure/waves (V wave), PMI, RV lift, S1, S2(P2), S3, S4, or edema.
Abdomen. Distention, flank bulging, fluid wave, bruits, rubs, liver size, tenderness, spleen, abdominal tenderness, or masses.
Rectal. Hemorrhoids, masses, and stool guaiac.
Neurologic. Asterixis, memory, orientation, or cerebellar signs.Laboratory
Minimal Lab: CBC with differential, reticulocyte and platelet count, Chem profile including ALT, AST, LDH, Alk Phos, Choleserol, urinalysis, chest x-ray.
Additional Lab: HBsAg/Aby, HAVAB IgM, Anti-HBc, Anti HCV, HD Ag,RPR, Mono Spot, PT, PTT, iron/TIBC, ferritin. ECG with heart failure. Consider ultrasound of gallbladder and bile ducts, HIDA scan, CT or liver/spleen scan.
- Hemolysis. Usual finding in patients with sickle syndrome is elevation of the indirect bilirubin < 4 mg.%, normal direct bilirubin, increased LDH, and (-) urine bilirubin. AST may be elevated from hemolysis but increased ALT is unusual. Most patients have relatively stable levels over time unless there are complications. If there are significant changes in these parameters, the cause of increased hemolysis must be determined. Normal direct bilirubin, ALT, and alkaline phosphatase are the rule without complications.
- Hepatic Congestion - Sequestration. Can occur from sequestration of sickle cells or right heart failure. Findings include increasing liver size and tenderness, increase in the direct and indirect bilirubin, increased alkaline phosphatase with less change in the AST or ALT. Acute hepatic sequestration can be a life threatening complication in adult patients with sickle syndromes characterized by rapid enlargement of the liver, severe anemia, hepatic and renal dysfunction, and other organ failure. With right heart failure there will be increased neck veins, and may be a RV lift, findings of tricuspid insuficiency, S3, edema, and ascites.
- Cholelithiasis. Presents with RUQ pain, nausea, and vomiting. Fever and leukocytosis suggests cholangitis (See Abdominal Pain,). Findings in obstructive jaundice include itching, elevation of alkaline phosphatase and bilirubin out of proportion to AST elevations, and increased prothrombin time which corrects with vitamin K. Ultrasound may document stones and HIDA scan is usually diagnostic. Previous cholecystectomy makes obstruction from stones less likely but not impossible. Cholestasis without stones is caused by a number of medications and can be seen in children as a spontaneous complication.
- Hepatitis. Patient soften present with history of exposure (contact, transfusion, or toxin),prodrome, anorexia, or fever. The liver is enlarged and tender. Viruses that cause hepatitis have been classified as hepatitis A (HAV), B (HBV), C (HCV), delta (HDV), and E (HEV). However, in some individuals, infection with the Epstein-Barr virus (EBV) or cytomegalovirus (CMV) also results in acute hepatitis. Stigmata of chronic liver disease may be present. AST, ALT, LDH, alkaline phosphatase, direct and indirect bilirubin are all usually elevated.
- Alcoholic and other toxic hepatitis can present with similar physical and laboratory findings but can be suspected from history. In addition, AST is elevated more than ALT in alcoholic hepatitis. Liver biopsy may be required for definitive diagnosis.
- Hepatic Fibrosis / Cirrhosis. Findings may be absent. Advanced cases have the stigmata of chronic liver disease, variable liver size, ascites, low albumin, diffuse increase in gamma globulins, elevated PT resistant to vitamin K, and variable enzyme changes.
- Infiltration / Tumor. Tumors or other types of liver infiltration present with weight loss, cachexia, painless jaundice or palpable gallbladder, and/or hard nodular liver. Alkaline phosphatase and bilirubin commonly elevated with AST/ALT often normal. CT scan is usually helpful.
- Iron Overload. Seen in patients after transfusion with about 500cc of red cells per kilogram body weight. Hepatitis picture is present with increased skin pigment, diabetes, cardiac failure. High serum iron and saturation and very high ferritin are characteristic.
- Hemolysis. No treatment necessary unless anemia or rate of hemolysis is increasing (See anemia).
- Hepatic Congestion - Sequestration. If the liver size or hemoglobin level is acutely changing, admission for transfusion and observation is indicated. Right heart failure may require chronic transfusion in most cases.(See Sequestration)
- Cholelithiasis. Admission usually indicated for observation if bilirubin is increasing from obstructive jaundice. HIDA scan or ultrasound are indicated to determine if there is common bile duct obstruction. Surgery consultation is indicated, because urgent surgery is often indicated for decompression and common duct exploration.
- Hepatitis. Acute viral hepatitis should be treated with a good diet, bed rest, and hydration. Patients with pain crisis, severe anorexia, or dehydration should be admitted. The rate of erythropoiesis may decrease with hepatitis, causing a rapid fall in hemoglobin level. Reticulocyte count and hemoglobin level should be monitored. Interferon therapy may be beneficial for chronic hepatitis C, especially in iron overloaded patients. The role of combination therapy in sickle cell disease is presently not defined.
- Hepatic Fibrosis / Cirrhosis. Admission is only necessary for hepatic decompensation, pain crisis, or other complications. Stop administration of all potential liver toxins such as alcohol, , acetaminophen, and use general support measures for liver patients. Should have liver biopsy to document etiology of liver dysfunction and define the prognosis.
- Infiltration / Tumor. Admit for diagnostic studies and liver biopsy if these conditions are suspected.
- Iron Overload. Iron studies and ferritin will support suspicion of increased iron burden. Liver biopsy is necessary to confirm increased iron burden and document the need for chelation. Presently the only treatment option is chronic subcutaneous or intravenous deferoxamine administration by portable pump over 12 hours at least five days a week. See Iron overload and chelation chapter.
Prevention of acute cholecystitis and common duct obstruction can be accomplished by screening for gall stones and performing cholecystectomy when stones are detected. Some individuals advocate this approach. Others feel that stones should not be removed unless symptoms are present so detection is deferred until symptoms arise.
Treatment of close contacts of cases of hepatitis A will decrease the incidence and severity of infection. Individuals that are to receive regular transfusions should be immunized with hepatitis B vaccine. HBIG immune globulin is given for know blood exposure, sexual contacts of acute cases, and neonatal exposure.
Prevention of iron overload is accomplished by judicious use of iron and transfusions in patients with sickle syndromes. Iron deficiency should be documented before chronic iron therapy is initiated. Ferritin levels should guide therapy so that iron supplementation is discontinued when stores are repleted. Transfusions should be given for established indications and symptoms that are clearly related to the anemia.
Patient and Parent Education
Patients and parents need to be reassured that the jaundice is a normal occurrence in sickle syndromes and the cause needs to be explained. They also need to be instructed to seek medical evaluation if there is significant change in the intensity of the jaundice.
The patient and their family needs to understand the indications and abuses of iron preparations and transfusion therapy so that they can become informed partners in making treatment decisions. The potential risks of hepatitis, alloimmunization, and iron overload must be presented with the potential benefits of transfusion.
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|Last Updated on Monday, 14 June 2010 14:20|