Celiac Hepatopathy 2019

A recent retrospective study (E Benelli et al. JPGN 2019; 68: 547-51) examines a large cohort of patients (=700) who were diagnosed with celiac disease (CD) from 2010-2016 and had available liver transaminases.

Key findings:

  • ALT values >40 U/L were elevated in only 3.9% (27/700)
  • Younger age (<4.27 years) correlated with a higher risk of liver involvement with OR 3.73
  • Of these 27 patients with elevated ALT, 18 had adequate followup.  All but 3 patients normalized ALT values after at least 1 year; of these, 1 was diagnosed with sclerosing cholangitis. In the other two, one was thought to be nonadherent with gluten-free diet and one had dropped ALT to 47 U/L.
  • Thus, definitive autoimmune liver disease was identified in only one patient

My take: This study shows a lower rate of liver involvement than previous studies.

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Joshua Tree National Park


Mauriac Syndrome (Glycogenic Hepatopathy)

A case report (T Malikowski et al. Gastroenterol 2017; 152: 947-49) provides some insight into a fairly common problem –elevated liver tests in the setting of poorly controlled type 1 diabetes mellitus.  This 18-year-old had presented with a glucose of 497 mg/dL, elevated lactate, aspartate aminotransferase 257 U/L, and alanine aminotransferase 178 U/L.

The authors note that Mauriac syndrome “occurs in young patients as a result of poorly controlled type 1 diabetes mellitus.”  It may result in growth retardation, pubertal delay, and cushingoid features.

“Glycogenic hepatopathy is a underrecognized complication of Mauriac syndrome that presents with abdominal pain, nausea, vomiting, elevated serum transaminases, elevated plasma lactate levels, and hepatomegaly  The pathogenesis stems from an accumulation of glycogen in the liver…The diagnosis…is made …when all other causes of liver disease have been excluded…When glucose control is achieved, prognosis is excellent.”

My take: There are many potential reasons for elevated liver enzymes associated with type 1 diabetes mellitus, including celiac disease, and autoimmune hepatitis.  However, familiarity with glycogenic hepatopathy helps with pattern recognition and helps explain the frequent concurrence of liver disease with poorly controlled type 1 diabetes mellitus.

Celiac Hepatopathies 2016

A recent study (GJ Lee et al. JPGN 2016; 63: 340-3) adds a little bit more information regarding hypertransaminasemia in newly diagnosed celiac disease.  Some previous information was summarized in a previous blog: Celiac Hepatopathies (2013)

In this retrospective, single center study, 185 children had transaminases obtained at the time of celiac diagnosis (185/388 = 47.7%).

Key findings:

  • Among this group, 28 (15.1%) had elevated transaminases, with an average of ALT 2.52 x ULN and AST 1.87 x ULN.
  • Patients with elevated liver transaminases tended to be younger (mean 6.3 yrs compared with 11.0 without elevation). Among those who had followup blood testing available, 15/21 (71.4%) normalized their values over an average of 210 days.
  • For the 6 who had persistent elevation of transaminases, 3 were suspected to have poor adherence, 1 was thought to have a fatty liver, 1 had only mild elevation, and 1 remained unexplained.

My take: This study indicates that elevated transaminases are common in children with celiac, particularly younger children.  As with other studies, the majority resolve on a gluten-free diet.  As there is a recognized association with autoimmune hepatitis, in those with elevated ALT, followup after institution of a gluten free diet seems prudent.

Iceberg Lake, Glacier Natl Park

Iceberg Lake, Glacier Natl Park