Cholestasis
Cholestasis is defined as a disorder of cholepoiesis and bile secretion as well as a mechanical or functional stoppage of the bile flow in intrahepatic or extrahepatic bile ducts — with bile components passing into the blood. • Cholestasis can occur both
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Page: Definition 236 Pathogenesis 236 Obstructive cholestasis 236 Non-obstructive cholestasis 237 Morphological changes 237 Forms of cholestasis 238 Causes of cholestasis 238 Extrahepatic obstructive cholestasis 238 238 Intrahepatic obstructive cholestasis Intrahepatic cholestasis 238 Genetically determined cholestasis 240 Primary storage diseases 240 Recurrent intrahepatic cholestasis in pregnancy 240 Benign recurrent intrahepatic cholestasis (BRIC) 241 ⫺ Aagenaes syndrome Progressive familial intrahepatic cholestasis (PFIC) 241 ⫺ Byler’s disease/syndrome Zellweger’s syndrome 242 242 Infantile Refsum’s syndrome Diagnosis 243 Anamnesis 243 Clinical findings 243 Fatigue 243 Pruritus and scratch marks 243 Xanthelasmas and xanthomas 243 Changes in biotransformation 244 Laboratory diagnostics 244 245 Imaging procedures Sonography 245 CT and MRI 246 ERC, PTC and EUS 246 Liver biopsy and laparoscopy 246 Clinical sequelae 248 Abdominal complaints 248 Steatorrhoea and diarrhoea 248 Malabsorption 248 Osteopathy 248 Renal dysfunction 248 Therapy 248 Mechanical cholestasis 248 Functional cholestasis 248 앫 References (1⫺82) 249 (Figures. 13.1⫺13.8; tables 13.1⫺13.11)
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13 Cholestasis 1 Definition Cholestasis is defined as a disorder of cholepoiesis and bile secretion as well as a mechanical or functional stoppage of the bile flow in intrahepatic or extrahepatic bile ducts ⫺ with bile components passing into the blood. • Cholestasis can occur both with and without jaundice. Morphology: The morphologist uses the term cholestasis to describe the presence of bile in the hepatocytes and hypertrophic Kupffer cells (⫽ cellular bilirubinostasis), particularly in the form of inspissated bile droplets and copper within the more or less dilated canaliculi (⫽ canalicular bilirubinostasis). • In extrahepatic cholestasis, bile is also found within the likewise mostly dilated interlobular bile ducts (⫽ ductular bilirubinostasis) as well as in the parenchyma in the form of “bile infarcts” or “bile lakes”. Pathophysiology: The biochemist defines cholestasis as a decrease in the secretion of bile as well as a reduction in the proportion of water, together with a respective effect on the substances dissolved in it. Clinical aspects: The clinician diagnoses cholestasis by the increase in bile acids, special enzymatic markers and cholesterol in the serum. 䉴 The principal biochemical symptom of cholestasis is the rise in bile acids in serum (as well as changes in its spectrum) in combination with an increase in enzymatic markers of cholestasis (AP, LAP, γ-GT, 5⬘-nucleotidase). Cholestasis is directly related to the metabolism of bile acids. • In clinical terms, the subsequent rise in activity of enzymatic markers of cholestasis may be attributed to cholestasis, yet these enzymes are not necessarily specific to this condition. (s. p. 107) 䉴 Dysfunction in the metabolism of bile ac
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