It is not for long term definitive treatment/cure, only as an adjunct (surgery is the aim for cure in most causes of Cushings syndrome). Metyrapone hence acts by inhibiting adrenal steroidogenesis. One side effect is hirsutism (in women) because of the excess androgen precursors created. The other commonly used agent for medical treatment of Cushings is ketoconazole (an anti-fungal agent). This does not exhibit the side effect of hirsutism. Pharmacology
Pharmacodynamics
Metyrapone blocks cortisol steroidogenesis by acting as a reversible inhibitor of 11β-hydroxylase. This stimulates adrenocorticotropic hormone (ACTH) secretion, which in turn increases plasma 11-deoxycortisol levels. Chemistry
Analogues of metyrapone include aminoglutethimide, amphenone B, and mitotane. Research
Metyrapone has been found in early human trials to reduce recollection of emotional memories in normal volunteers. The volunteers showed significant impairment in ability to retrieve memories with negative emotional content while not impairing memories with neutral content. This has significant implication in the study of the process of emotional healing in post traumatic stress disorder.Due to the permissive action of cortisol on glucagon, partial blockade of cortisol may reduce
the effects of circulating glucagon in chronically increasing blood glucose in metabolic syndrome (syndrome X) and type 2 diabetes. See also
ACTH stimulation test


== References ==
Congenital hepatic fibrosis  is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and fibrosis that do not alter hepatic lobular architecture. The fibrosis would affect resistance in portal veins leading to portal hypertension. Cause
The condition is usually congenital, but sporadic cases have also been reported. It may be associated with other congenital defects, commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation. Mechanism
Embryogenically, congenital hepatic fibrosis is due to malformation of the duct plate, a round structure appearing in the eighth week of gestation that is formed by primitive hepatocytes, which differentiate into cholangiocytes. Congenital hepatic fibrosis usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life. Clinical features may vary but commonly include cholangitis, hepatomegaly and signs of portal hypertension. Diagnosis
Liver biopsy is diagnostic. In biopsy there is diffuse periportal and perilobular fibrosis in broad bands, containing distorted duct like structure or microcyst formation. Management
Management includes control of esophageal bleeding/varices and treatment of associated renal disease if present. See also
Caroli disease
Polycystic kidney disease
Von Meyenburg complex
Biliary hamartomas

References
External links

GeneReviews/NCBI/NIH/UW entry on Congenital Hepatic Fibrosis Overview