Characterizing Severe Liver Disease with Cystic Fibrosis

A recent retrospective study (JR Stonebraker et al. Clin Gastroenterol Hepatol 2016; 14: 1207-15) examined data from 561 patients (76 international centers) with cystic fibrosis (CF), liver disease and portal hypertension.  The study period spanned 1999-2013. Key findings:

  • Male patients were diagnosed earlier: 10 vs 11 year, p =.01.  63% of patients were males.
  • Splenomegaly was noted in 99% and varices in 71%
  • Levels of liver enzymes were near normal in most patients. 63% had transaminases that were less than 2 x ULN.
  • 91 (16%) received liver transplants at a median age of 13.9 years.  Hallmarks of those receiving liver transplants included lower platelet counts (78 vs 113, P<.0001), higher INR (1.4 vs 1.2, P<.0001), and lower albumin (3.3 vs 3.7, P =.0002).
  • 99% of patients in this cohort were pancreatic insufficient

My take: In patients with CF, those with severe liver disease often present by age ~10 years.  Features of portal hypertension, like splenomegaly and low platelet counts, appear to be more important than liver enzymes.

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