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| Cirrhosis | ||
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Andrew E. Mulberg
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Database Data Gathering Physical Examination Laboratory Aids Therapy Common Questions and Answers Bibliography |
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DEFINITION
Cirrhosis is a process characterized by increased fibrous tissue and nodule formation following necrosis of the hepatocyte within the liver.
CAUSES
The number of etiologic agents is numerous and detailed below:
GENETICS
In several disorders, including sclerosing cholangitis and hemochromatosis, there have been identified HLA markers. However, no specific markers correlate with cirrhosis.
EPIDEMIOLOGY
Based on the varying etiologies, no specific epidemiologic pattern can be identified.
COMPLICATIONS
The final common pathways of hepatic dysfunction secondary to cirrhosis are related to hepatocyte and vascular insufficiency. These include encephalopathy, ascites, liver failure, and portal hypertension leading to hemorrhage.
PROGNOSIS
The prognosis for cirrhosis leading to decompensation depends on the etiologic agent. Poor prognostic features include elevated PT, ascites, gastrointestinal hemorrhage, encephalopathy, poor nutrition, hypoalbuminemia, and neurologic changes.
| DATA GATHERING | ||
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HISTORY
Based on the varying etiologic agents, one should elicit pertinent historical features characteristic of each specific problem as detailed:
| PHYSICAL EXAMINATION | ||
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Estimation of liver size may be helpful in suggesting cirrhosis. Helpful diagnostic clues include:
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TESTS
Imaging
There are various options available to the clinician for diagnosing cirrhosis of the liver.
| THERAPY | ||
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DRUGS
Diuretics and decreased sodium content of foods will enhance the resolution of ascites, a complication of cirrhosis.
DURATION
Medical therapy is performed until the clinical status requires advancement of therapy, including hepatic transplantation or stabilization of clinical status.
SURGICAL
For management of portal hypertension, the diversion of portal blood flow to the systemic system can be established but is associated with complications and increased morbidity and mortality. Varying shunt procedures include mesocaval, portocaval, and distal splenorenal shunts. In the setting of decompensated cirrhosis, hepatic transplantation may be necessary.
| COMMON QUESTIONS AND ANSWERS | ||
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Q: Will my child with cystic fibrosis develop cirrhosis?
A: The medical literature cite a 5% to 20% incidence of cirrhosis in children with cystic fibrosis. Many factors seem to relate to the development of cirrhosis in these children, but the genetic type of cystic fibrosis does not seem to be a cause only.
Q: Will every child with cirrhosis need a liver transplant?
A: Most children who develop cirrhosis from causes such as biliary atresia or metabolic disease will ultimately require a liver transplant.
ICD-9-CD 571.0
| BIBLIOGRAPHY | ||
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Ernst O, Gottrand F, Calvo M, Michaud L, Sergent G, Mizrahi D, L’Hermine C. Congenital hepatic fibrosis: findings at MR cholangiopan-creatography. Am J Roentgenol 1998;170(2):409–12.
Perisic VN. Long-term studies on congenital hepatic fibrosis in children. Acta Paediatr 1995;84(6):695–696.
Sherlock S, Dooley J. Hepatic cirrhosis. In: Sherlock S, Dooley J, eds. Diseases of the liver and biliary system, 9th ed. London: Blackwell Science, 1993: 357–369.
Copyright © 2000 Lippincott Williams & Wilkins
M. William Schwartz, Louis M. Bell, Jr., Peter M. Bingham, Esther K. Chung, David F. Friedman and Andrew E. Mulberg, The 5 Minute Pediatric Consult