Laennec's cirrhosis

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB =
ICD10 =
ICD9 = ICD9|571.2
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID =

Laennec's cirrhosis is named after René Laennec,cite journal |author=Duffin JM |title=Why does cirrhosis belong to Laennec? |journal=CMAJ |volume=137 |issue=5 |pages=393–6 |year=1987 |month=September |pmid=3304599 |pmc=1492806 |doi= |url=] a French physician and the inventor of the stethoscope. It is a disease of the liver in which the normal lobular architecture is lost, with fibrosis and later nodular regeneration. Laennec's cirrhosis can be associated with inflammatory polyarthritis, most commonly affecting the shoulders, elbows and knees. Osteoporosis, soft tissue swelling in peripheral joints and sometimes calcific periathritis are seen.

In the developed world, Laennec's cirrhosis most commonly affects middle-aged males, typically ages 40-60. This is the most common form of cirrhosis in the U.S.

In areas of the world afflicted with chronic starvation, (Africa and Asia), children are most commonly afflicted.

Etiology

Severe protein deficiency can cause Laennec's cirrhosis.Two causes have been identified. The first is malnutrition, or, more specifically, protein deprivation. This is seen in starving children who have insufficient supplies of protein and therefore manufacture insufficient amounts of lipoproteins. They develop fatty livers: it is presumed that if they survive, cirrhosis will develop.

Chronic alcoholism can cause Laennec's cirrhosis.The second is alcohol. Whether or not alcohol alone can produce fatty nutritional cirrhosis has been debated for decades. Current evidence is that it can. If so, the condition should be renamed "alcoholic cirrhosis." Those who do not subscribe to the "alcohol-as-a-poison" school state that the changes to be described are the result of malnutrition common to alcoholics. They argue that alcoholics, in a sense, are no different than starving African children -- both have protein deprivation.

tages

Laennec's cirrhosis has three stages.The pathologic features of this form of cirrhosis change with time. Therefore, it is helpful to break the disease down into three stages: 1) the fatty liver stage, 2) the fibrotic liver stage and 3) the nodular liver stage.

In early stages the liver is large and fatty.In this early stage, fat accumulates in the liver cells around the central vein (fatty change). The liver becomes large, even huge (hepatomegaly). The normal liver weighs about 1,200 grams. By comparison, fatty livers can weigh in at over 6,000 grams and may, in the living patient, fill the abdominal cavity (remember that the normal liver extends 2-3 finger breadths below the right costal margin). At autopsy, the fatty liver is greasy and a cut surface is yellow. As dramatic as these changes are, the fatty change is reversible.

In later stages, the liver becomes scarred (fibrotic).In this stage, the liver returns to a more normal size; however, it does not return to normal in any other way. In fact, the changes that develop during this stage are irreversible. The fatty change subsides and is replaced by fibrosis (scarring) and some chronic inflammation. No doubt the retreat of fatty change and the shrinking effect of scar tissue is responsible for the over-all decrease in liver size.

In the final stage, the liver becomes lumpy (nodular).In this stage, the liver shrink even further. It may not extend below the costal margin at all. Liver cells attempt to regenerate in an increasingly fibrotic setting. They find it difficult to do so and form "regenerative nodules" that only partially carry out normal liver function. This shrunken, nodular texture has been dubbed the "hob-nail" or "cobble stone" effect.

References