Moffitt AM Report 5/16/17: Liver Involvement and Turner Syndrome

 

Hello Moffitt!

We discussed 2 interesting cases today during Morning Report.

Case 1: Thanks Sam Miller, for presenting the case of a middle aged woman with palpitations and presyncope, found to have significant T wave inversions and stress cardiomyopathy!

Case 2: Thanks Mike Healy, for presenting the case of a woman with Turner Syndrome and LFT abnormalities.

Love,

SamMy

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TAKE HOME POINTS

  • Liver involvement in Turner Syndrome is frequent, and ranges from steatosis to cirrhosis to biliary involvement!
  • Takotsubo cardiomyopathy is a diagnosis of exclusion that is characterized by regional systolic dysfunction of LV, mimicking an MI, but in the ABSENCE of angiographic evidence of obstructive CAD or plaque rupture.

LIVER INVOLVEMENT IN TURNER SYNDROME

  • A Brief note on Turner Syndrome:
    • It is one of the most frequent inherited diseases, with prevalence of 1/2500 female live birth
    • Results from total or partial loss of X chromosome
    • Syndromic findings include short stature, gonadal dysgenesia, dysmorphic features, congenital heart and renal malformations, and predisposition to autoimmune diseases

 

  • Liver involvement is frequent in Turner syndrome (20-80% prevalence of liver test abnormalities)
  • The exact pathophysiology behind elevation of liver enzymes is not completely understood, but excess weight and estrogen replacement therapy have been proposed as causative factors.
  • Various hepatic changes occur in Turner Syndrome
      • Steatosis and non-alcoholic fatty liver disease: thought to be secondary to insulin resistance and obesity associated with Turner.
      • Architectural changes (cirrhosis, nodular regenerative hyperplasia, multiple focal nodular hyperplasia): Thought to be a form of adaptation to microcirculatory disturbances causing heterogeneous distribution of intrahepatic blood flow
      • Biliary involvement : Several types of biliary involvement reported in Turner syndrome, including sclerosing cholangitis, PBC, and biliary atresia : thought to be related to autoimmune diathesis in Turner Syndrome

pic 1a

  • Prognosis: Studies are limited, but major liver complications are uncommon, and are only observed in select cases of marked architectural changes

 

 

CHECK OUT OUR PRIOR PEARLS ON TAKOTSUBO CARDIOMYOPATHY

https://ucsfmed.wordpress.com/2016/08/30/takotsubo-cardiomyopathy/

Evernote Link: https://www.evernote.com/shard/s338/sh/597a78e8-62f0-442e-a9d5-a6109d2c009a/dde267a829c6b1f3642a5f628c62d99d

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