Cirrhosis micronodular

Fig. 31.24 Haemochromatotic cirrhosis micronodular, slate-grey to brownish discolouration, rich in septate fibrosis, marked neovascularization...

Hepatic cirrhosis is typically the end stage of liver disease. Cirrhosis describes an irreversible change (Treinen-Moslen, 2001) characterized by accumulation of excessive collagen deposition in the form of bridging fibrosis which disrupts the hepatic architecture. Cirrhosis may be micronodular or macronodular depending on the amount of fibrosis and tissue regeneration. Liver transplantation is the only solution to restore adequate liver fimction in human medicine. 

Fig. 7.15 Active, micronodular, in part moderately nodular (alcohol-related) cirrhosis with pronounced pathways of postnecrotic connective tissue...

Fig. 7.16 Inactive, micronodular (drug-related) cirrhosis with fine pathways of connective tissue clinically and histologically definable as liver fibrosis (sonographically no signs of portal hypertension)...

There are no morphological differences between alcoholic steatohepatitis and NASH. In some 30(-50)% of patients with NASH, predominantly micronodular cirrhosis develops within 5 years. 

UVexaminations (366 nm), to which every liver bioptate should be subjected, the red fluorescence has different intensities, facilitating classification into PCT groups A-D types A and B (no clinical symptoms) show dotlike fluorescence, type C (latent PCT) has mostly reticular fluorescence, and type D (manifest PCT) is characterized by homogeneous red fluorescence, (s. fig. 7.10 ) (s. pp 145, 158) In the further course, scar tissue or micronodular cirrhosis develop. There is a greater risk of hepatocellular carcinoma (15-25%) (due to concomitant HBV or HCV infection as well as haemochro-matosis ). (305, 309-311)... 

Fig. 31.21 Explan ted liver with micronodular cirrhosis in Wilson s disease (18-year-old woman presenting with acute liver failure) (Sirius)...

The morphological spectrum may therefore range from steatosis, acute hepatitis, fulminant course, chronic hepatitis, aggressive episodes in chronic hepatitis and liver fibrosis through to micronodular cirrhosis. Complete cirrhosis can already exist in children aged 4-5 years. The development of hepatocellular carcinoma is extremely rare (360) it is assumed that copper has a protective effect against malignant transformation. (391,393)... 

If it is not possible to remove the obstruction and achieve defect healing (i.e. with fibrous residues) of the chronic (relapsing) cholangitis, the inflammatory destruction of periportal liver parenchyma will result in portoportal bridge formations and thus isolation of hepatic lobules by means of connective tissue. Monolobular, mostly micronodular biliary cirrhosis develops. (4l, 54) (s. fig. 32.5)... 

Fig. 32.5 Biliary cirrhosis following chronic relapsing and abscess-forming cholangitis green and grey dirty colouration of the deformed micronodular surface. (Chronic cholecystitis with formation of a shrunken gall bladder)...

While the formation of nodes is a typical finding on the liver surface (s. figs. 28.13 35.2-35.4), in sectional preparations (s. fig. 35.5) and under the microscope (s. fig. 35.4), it is not an obligate criterion. It is hardly recognizable in many cases, such as in haemochromatotic, biliary and alcoholic cirrhosis. Smooth cirrhosis, showing a smooth liver surface despite complete transformation, is likewise found it is micronodular and poor in fibres, (s. fig. 14.3)... 

Micronodular cirrhosis Monolobular (monoacinar) cirrhosis consists of individual hepatic lobules separated by connective tissue, with the central vein maintained in the interior. In pseudolobular cirrhosis, no elements of the lobular architecture and no central vein can be detected the nodules in this type of cirrhosis are small. The monolobular and pseudolobular forms are classified as micronodular cirrhosis, or Nagayo type C (with node size <0.3 cm). A striking feature is the uniformity of the nodules, (s. figs. 35.4, 35.5)... 

Fig. 35.4 Complete, micronodular, progressive liver cirrhosis with formation of pseudoacini (Sirius red)...

The micronodular form can develop into a macronodular form, but not vice versa. Generally, micronodular cirrhosis contains more fibrosis than parenchyma therefore it is firmer than macronodular cirrhosis. The development of micronodular cirrhosis, e. g. in alcohol abuse, may be caused by the fact that continuous alcohol intake blocks the protein synthesis, and thus the parenchyma has no time or chance for cellular proliferation. This form of cirrhosis mainly results from alcohol abuse, but may also be found in haemochromatosis,... 

Fig. 35.5 Complete, micronodular to medium-nodular liver cirrhosis after chronic viral hepatitis B (section, native preparation)...

Mixed-nodular cirrhosis is considered to be a transitional form between micronodular and macronodular cirrhosis (V.J. Desmet et al., 1990). Due to variations in the regenerative capacity of the cirrhotic liver, 50% of... 

Type of cirrhosis Macronodular cirrhosis (which is usually a posthepatitic form of cirrhosis) is the type most often associated with HCC (about 55% of cases). Compared to micronodular cirrhosis (usually alcohol-induced), development of HCC must be anticipated in 5-10%o of patients. In this context, the actual presence of coarse-nodular cirrhosis seems to pose a greater risk of HCC than the aetiology of the underlying cirrhosis. The adenomatous and hyperplastic transformations frequently found in cirrhosis tend to develop into HCC. 

Histology provides useful diagnostic evidence. Sinusoidal endothelial damage can be found, including extra-vascular accumulation of erythrocytes in Disse s spaces as well as subendothelial oedema and cellulation. After 2 to 3 days, delicate fibres appear within the central and sublobular veins, occasionally also in the medium-sized hepatic veins, ultimately resulting in occlusion of the lumen. Fibrotic thickening of the vessel walls occurs. Stenosis and thrombosis of the small hepatic veins cause extensive sinusoidal congestion. The liver cells become necrotic or atrophic. Micronodular cirrhosis develops in a chronic course, (s. fig. 29.10)... 

Parenchjmal hver damage can occur in patients taking tiabendazole and abnormal liver function tests have been documented (9). There have been well-studied cases of bile duct injury, which can lead to micronodular cirrhosis (10), and a case in which these various forms of liver disorder co-existed and hver transplantation proved necessary (11). 

Patient 20 has been shown to be mosaic for a PEX6 defect (Moser, 1999). Cultured skin fibroblasts and liver biopsy samples have demonstrated a mixture of normal and abnormal cells (Pineda, et al., 1999). The patient is now 16 yr old. Developmental milestones were delayed at 2 yr. Liver biopsy showed micronodular cirrhosis. Neurologic exam at 3.5 yr showed nystagmus, head tremor, and generalized spasticity, and he was unable to walk. At 4 yr of age, he was started on a diet containing medium-chain triglycerides, pure olive oil (20% of daily fat intake), and vitamin A supplementation. This coincided with clinical improvement as evidence by the disappearance of abnormal eye movements and head tremor and reduction of spasticity. This improvement was attributed to a reduction in phytanic acid levels. Visual evoked responses improved between 4 and 6 yr of age. DHA therapy was added to the regimen at age 6.7 yr. Further improvement of the visual evoked responses occurred. At 12 yr, motor and mental improvements have been maintained. He is able to stand with support. His IQ is 60 and he attends a special school. 

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