Cirrhosis
Cirrhosis comes from the Greek word meaning tawny. It is a condition where there is abnormality of the architecture of the liver, with fibrous strands between the portal tracts and lumpy areas (called nodular) of regeneration of the liver cells between the strands. While it used to be that the regeneration, and therefore the cirrhosis, was described as either micro-nodular or macro-nodular depending on whether the lumps were greater or smaller than 3mm, it is now realised that a better classification is according to whatever has caused the disease. It develops over a prolonged period of time as a result of damage to, and death of, liver cells, which produce the fibrous scarring, and subsequent regeneration and attempts at repair.
There are many different causes in childhood and adolescence, from the effects of infection with hepatitis B or C; metabolic disorders such as Wilson's disease, obstructive disorders affecting the outflow of bile as in cystic fibrosis - all described elsewhere in this section - or chronically increased pressure in the blood vessels leading from the liver, as occurs in chronic cardiac failure (see the heart in the chest cavity section). While cirrhosis, by its very nature and the timescale over which it develops, may be asymptomatic while the remaining liver tissue manages to fulfil its role and meet the needs of the metabolism, (where the cirrhosis is said to be compensated), as the disease progresses there are signs of decompensation, with jaundice (yellowing of the skin), ascites (free fluid in the abdominal cavity, causing swelling), encephalopathy (disturbance of brain function) and possibly haemorrhage in the gastro-intestinal tract as a result of portal hypertension.
Cirrhosis cannot be treated, but its progression can be halted in cases where the causative agent can be removed, but in many cases this is not possible. In some metabolic or autoimmune causes treatment with in used immunosuppressants agents including steroids may be beneficial, but in many cases the only treatment available is liver transplantation.
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