Hepatobiliary disease, and

Deficiency develops in patients with severe fat malabsorption, cystic fibrosis, chronic cholestatic hepatobiliary disease, and in two rare groups of patients with genetic diseases ... 

Elevated LAP values are found predominantly in biliary and cholestatic diseases - in accordance with AP. In liver diseases due to alcohol abuse, LAP values are exhibited both more frequently and with higher values than AP. In hepatitis mononucleosa, LAP is generally also more clearly elevated than AP. Significant increases in LAP are found in pancreatic and breast cancer as well as in collagenoses of the vascular type. LAP is not found in bone there is no evidence of elevated LAP in bone diseases. Normal LAP in connection with an increase in AP consequently rules out hepatobiliary diseases and requires further investigation. In these cases, parallel determination of AP and LAP is advisable. 

Tab. 5.20 Autoimmune-associated hepatobiliary diseases and their possible autoantibody constellations (s. tab. 33.1)...

Elevations in serum ALP activity commonly originate from one or both of two sources the liver and bone. Consequently, serum ALP measurements are of particular interest in the investigation of two groups of conditions hepatobiliary disease and bone disease associated with increased osteoblastic activity (see Bone Enzymes section later in this chapter). 

Assay of NTP activity has been considered of value as an addition to measurement of nonspecific total ALP in patients with suspected hepatobiliary disease, and abnormal NTP activity is routinely interpreted as evidence of a hepatic origin of increased ALP activity in serum. However, approximately half of individuals in whom liver ALP activity is increased in serum may simultaneously show a normal NTP. On the other hand, increased NTP in the serum of patients with normal liver ALP is very often associated widi the presence of liver disease. Thus the frequent dissociation of the two enzyme activities supports the utility of determining both (fiver) ALP and NTP to increase the diagnostic efficiency for diseases of the liver. 

Batsakis, G. Sodeman, T. A. and Deegan, M. J. Enzymatic evaluation of hepatobiliary disease. Laboratory Med. 

Hepatobiliary disease occurs due to bile duct obstruction from abnormal bile composition and flow. Hepatomegaly, splenomegaly, and cholecystitis may be present. Hepatic steatosis may also be present due to effects of malnutrition. The progression from cholestasis (impaired bile flow) to portal fibrosis and to focal and multilobar cirrhosis, esophageal varices, and portal hypertension takes several years. Many patients are compensated and asymptomatic but maybe susceptible to acute decompensation in the event of extrinsic hepatic insult from viruses, medications, or other factors.7... 

Serum aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, y-glutamyl transferase, and bilirubin may be elevated in patients with hepatobiliary disease. 

Plasma was obtained from ten healthy fasting adults (44-70 years) who do not have any hepatobiliary or gastrointestinal disease, and then stored frozen at -20°C until analysis. 

GGT is fouud particularly iu hepatocytes aud biliary epithelial cells. GGT serum levels may be high iu liver disease, but it is particularly a feature of biliary outflow obstruction more so than hepatocellular damage. GGT serum measuremeut provides a very sensitive indicator of the presence or absence of hepatobiliary disease. However, raised GGT levels have also been reported in a variety of other clinical conditions, including pancreatic disease, myocardial infarction, chronic obstructive pulmonary disease, renal failure, diabetes, obesity and alcoholism. It is also a sensitive indicator of liver damage through alcohol iugestion. 

Figure 9.88 Separation of uridine from UMP and blood components. (A, B) Chromatograms of UMP in the reaction mixture without enzyme and uridine standard (50 fiM), respectively. Samples were incubated with (C, E, G, /) or without (D, F, H, J) UMP. QD) Erythrocytes from a normal subject. ( , F) Erythrocytes from a lead-poisoned subject. (G, H) Plasma from a person suffering from hepatobiliary disease. (From Sakai et al., 1982.)...

Liver disease is now recognised as a major complication of type 2 diabetes. Diabetes mellitus can lead to metabolic changes that alter normal hepatic and biliary function and structure. Type 2 diabetes is associated with an increased risk of a range of hepatobiliary diseases, including non-alcoholic fatty liver disease, cirrhosis, acute liver failure, hepatocellular carcinoma and cholelithiasis [22]. 

Brunt EM. Nonalcoholic steatohepatitis. Semin Liver Dis 2004 24 3-20. Marchesini G, Bugianesi E, Forlani G, et al. Nonalcoholic fatty liver, steatohepatitis, and the metabolic syndrome. Hepatology 2003 37 917-923. Wanless IR, Lentz JS. Eatty liver hepatitis (steatohepatitis) and obesity an autopsy study with analysis of risk factors. Hepatology 1990 12 1106-1110. Zimmerman HJ. Hepatotoxicity. The adverse effects of drugs and other chemicals in the liver, 2nd edn. Philadelphia Lippincott Williams Wilkins, 1999. Mason P. Cystic fibrosis - the disease. Hosptal Pharmacist 2005 12 201-207. Tolman KG, Eonseca V, Tan MH, et al. Narrative review hepatobiliary disease in type 2 diabetes mellitus. Ann Intern Med 2004 141 946-956. 

CT is a technique that uses ionising radiation and computer processing to generate cross-sectional three-dimensional images of the internal organs. In assessment of the hepatobiliary tract both an oral contrast agent and an intravenous contrast medium are required to visualise the bowel and blood vessels, respectively. In the context of hepatobiliary disease, CT is particularly useful in assessing the extent of mass lesions. 

Determining whether there has been any history of hepatobiliary disease in the family and establishing the geographic and ethnic origin of the patient are key anamnestic factors. It must be ascertained whether the disease has occurred suddenly, developed gradually, or simply not been noticed up to now. It is also important to determine whether this is a primary or secondary liver disorder (the latter is a concomitant reaction which has occurred during a systemic illness or other organic disease). The anamnestic search for the cause of the infection in the patient takes into account viral, bacterial, parasitic and mycotic diseases, (see chapters 22-26) (s. tab. 4.1)... 

Hepatobiliary enzymes Each organ possesses a typical quantitative and, to some extent, qualitative distribution of enzymes resulting in a cellular profile of enzymes, which is termed the enzyme pattern. Thus within the hepatocytes and the biliary ducts, the liver also possesses a characteristic distribution of enzymes with gradually varying specificity. This specificity of essential hepatobiliary enzymes is of importance for the diagnosis of hepatobiliary diseases. (26) (s. tab. 5.4)... 

In hepatobiliary diseases, the sensitivity and specificity of y-GT is 95%, since y-GT is also located in cholangiocytes. In individuals with a healthy liver, the specificity is 74%, in overall healthy individuals it is 95%. In cholestasis, the sensitivity of y-GT is six times that of AP and nine times that of LAP Obstruction of bile flow can be the cause of isolated y-GT elevation in fatty liver. 

The Increase In AP activity is stimulated by bile acids. A rise in bile acids, which is considered to be the most sensitive and earliest marker of cholestasis, precedes any elevation in AP. The latter derives from enzyme synthesis with increased secretion into the blood. Under pathological conditions, bile duct AP is formed, which is a sensitive marker for hepatobiliary diseases, cholestasis and space-occupying lesions of the liver. The sensitivity is 80-100% in cholestatic diseases. AP activity is usually higher in obstructive jaundice and cholangitis than in intrahepatic obstructions, and it is highest in the vanishing bile duct disease or in complete obstruction. (13, 39, 41) (s. tabs. 5.9 13.2-13.4)... 

Tab. 5.19 Autoantibodies and their subtypes in chronic hepatobiliary diseases...

From a biochemical viewpoint, three forms of activity can be differentiated in hepatobiliary diseases (7.) enzymatic activity, (2.) mesenchymal activity, and (i.) serological and immunological activity, (s. tab. 5.22)... 

Tab. 7.1 Morphological examination methods and additional techniques for the clarification of hepatobiliary diseases...

Attention must be paid to severe clotting disorders, cardiac and coronary insufficiency, severe cardiac arrhythmia, serious hypertension, respiratory insufficiency and purulent peritonitis as possible contraindications. The same is true of Bekhterev s disease and cerebral insufficiency (depending on the respective severity). Despite a wide range of indications, the list of contraindications (with the exception of hepatogenic clotting disorders) only covers severe extrahepatic diseases. In these cases, it is the treatment of the serious condition which is of paramount importance and not the diagnostic clarification of abdominal or hepatobiliary diseases. (228, 244, 254, 266, 275) (s. tab. 7.13)... 

Acute tubular necrosis can occur in the course of a hepatobiliary disease. In an aetiopathogenetical context, hypoxia, hypotension, nephrotoxins and so far undefined biochemical substances are deemed responsible. The outcome is a disruption in the reabsorption of sodium and water the urine is less concentrated (isosthenuria). There is a greater excretion of sodium (> 30 mEq/1) and of beta-2 microglobulin in the urine. Acute, yet in principle reversible renal failure can develop. Therapy thus consists of bridging the phase of insufficiency temporarily by dialysis. 

Fibrosis is usually the consequence or concomitant symptom of a chronic hepatobiliary disease, the course of which can itself in turn be unfavourably influenced by the fibrosis. The matrix substances, which are being produced in greater quantities, are increasingly deposited in Disse s space in the portal field and periportal area as well as around the terminal liver vein. 

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