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What all does the liver synthesize?
- Proteins
- Coagulation factors
- Ammonia
- Carbs
- Fat
- Ketones
- Vit A
- Enzymes
- and so on...
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What is the principal pigment in bile that is derived from hemoglobin breakdown?
Bilirubin
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How does bilirubin get transported to the liver?
- Bilirubin forms a complex with albumin for transport to the liver
- In this form, bilirubin is unconjugated and NOT water soluble
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When is bilirubin conjugated?
- Conjugated in the hepatocyte endoplasmic reticulum with glucuronic acid to form bilirubin diglucuronide
- The reaction is catalyzed by uridine diphosphate (UDP) glycuronyltransferase
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Which form of bilirubin is water soluble, unconjugated or conjugated?
Conjugated is water soluble
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What has an orange-brown pigment that gives stool its characteristic color?
Urobilin
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What does the liver secrete to assist in digestion?
Bile
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Where is bile stored?
Gallbladder
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What organ is the primary site in the body for synthesis of waste products, conjugation of hormones, and bilirubin to water-soluble forms, and conversion of drugs to metabolites for excretion in urine or stool?
Liver
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What clinical condition occurs when there is excessive erythrocyte destruction (as hemolytic anemias, spherocytosis, toxic conditions, hemolytic disease of the newborn caused by Rh or ABO incompatibility)?
Prehepatic jaundice
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What clinical condition occurs when the liver cells malfunction and cannot take up, conjugate, or secrete bilirubin?
Hepatic jaundice
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What clinical condition:
Defect in the ability of hepatocytes to take up bilirubin
Due to transport problem of bilirubin from the sinusoidal membrane to the mircrosomal region
Gilbert syndrome
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What clinical condition is a partial or complete deficiency of UDP-glycuronyltransferase?
Crigler-Najjar disease
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What clinical condition is characterized by a defective liver cell excretion of bilirubin due to impaired transport in the hepatocyte of conjugated bilirubin from microsomoal region to the bile canaliculi?
Dubin-Johnson syndrome
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What clinical condition is characterized by a level of UDP-glycuronyltransferase is low at birth?
Neonatal physiological jaundice
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What clinical condition may be caused by hepatocyte injury such as cirrhosis, bile duct injury (like Rotor syndrom or neoplasms)?
Intrahepatic jaundice
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What clinical condition occurs when and obstruction blocks the flow of bile into the intestines?
- Posthepatic jaundice
- (also called extrahepatic cholestasis)
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What disorder is a result of chronic scarring of liver tissue turning it into nodules?
Cirrhosis
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What is the tumor that is the primary cancer of the liver?
Hepatocellular carcinoma or hepatoma
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What tumor arises from other cancerous tissue where the primary site was of lung, pancreas, gastrointestinal tract or ovary origin?
Metastatic liver tumors
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What syndrome has an unknown cause but include symptoms of encephalopathy, neurologic abnormalities including seizures or coma, and abnormal liver function tests due to hepatic destruction?
Reyes syndrome
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What lab tests are markers for hepatocellular necrosis?
- ALT: Most specific for hepatocyte injury
- AST: Less specific than ALT
- LD: Least specific
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What lab tests are markers that reflect cholestasis?
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What other lab tests are used to assess liver disorders?
- Bilirubin (total, direct (conjugated), and indirect (unconjugated))
- Albumin
- Ammonia
- AFP
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What test methodologies are used to measure bilirubin?
- Jendrassik-Grof total bilirubin test
- Direct spectrophotometric
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What are the reference ranges for bilirubin?
- Infants Total Bilirubin: 2-6 mg/dL
- Adults Total Bilirubin: 0.2-1.0 mg/dL
- Indirect Bilirubin: 0.2-0.8 mg/dL
- Direct Bilirubin: 0.0-0.2 mg/dL
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What causes a decrease in urobilinogen?
Posthepatic obstruction
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What causes an increase in urine urobilinogen?
- Hemolytic disease
- Hepatocellular disease (Hepatitis)
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What is the reference range of urine urobilinogen?
0.1-1.0 Ehrlich units/2 hr
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Deficiency of any of the specific enzymes that catalyze the formation of the prophyrinogens results in _________
Results in excess formation of the corresponding porphyrin
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Name the clinically significant porphyrins
- Urophorphyrin
- Coproporphyrin
- Protoporphyrin
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Name the types of porphyrias
- Plumboporphyria
- Acute intermittent porphyria
- Congenital erythropoietic porphyria
- Porphyria cutanea tarda
- Hepatoerythropoietic porphyria
- Herediatry coproporphyria
- Variegate porphyria
- Erythropoietic porphyria
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What is the term for overproduction or accumulation of porphyrins and precursors in the bone marrow?
Erythropoietic porphyrias
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What is the term for overproduction or accumulation of porphyrins and precursors in the liver?
Hepatic porphyrias
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What test methodology is used to measure porphyrins?
- Watson-Schwartz test
- (forms red condensation product with porphobilinogen)
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