Case Report

Glycogenic Hepatopathy: A Rare Hepatic Complication of Poorly Controlled Type 1 DM

Table 2

Comparison of celiac hepatopathy and glycogenic hepatopathy.

Celiac hepatopathy (CH) [10, 11]Glycogenic hepatopathy (GH)

ClinicallySymptoms: asymptomatic, mild symptoms commonly malaise and fatigue, or symptoms of severe liver failureSymptoms: mild epigastric pain, nausea, and vomiting
Signs: range from normal physical examination to liver failureSigns: hepatomegaly ± tenderness
Liver enzymes: mild to moderate (less than 5 times the upper normal limit)Liver enzymes: severe flares of transaminases reaching up to 2000–4000 U/L
Liver function panel: depends on the degree of liver injuryLiver function panel: normal

RadiologicalAbdominal ultrasound: normal to coarse echo texture associated with findings that suggest the activity or suspension of unrecognized CD asAbdominal ultrasound
Hepatomegaly measured around (17–23 cm) bright coarse echotexture
(i) dilated small bowel loopsAbdominal CT scan: hepatomegaly and high-density and marked attenuation
(ii) enlarged mesenteric lymph nodes
(iii) increased peristalsis
(iv) abnormal jejunum folds
(v) enlarged mesenteric lymph nodes

HistologicallyHistological findings:Histological findings:
(i) Nonspecific, most commonly periportal inflammation, mononuclear infiltration of the parenchyma, bile duct obstruction, hyperplasia of the Kupffer cells(i) Swollen hepatocytes and pale cytoplasm
(ii) Steatosis(ii) Abundant cytoplasmic glycogen deposits are demonstrated by periodic acid-Schiff (PAS) staining, and glycogen removal is demonstrated by diastase digestion
(iii) Less common, advanced lesions with fibrosis and liver cirrhosis. Fibrosis (all stages), and cirrhosis(iii) No evidence of necrosis, inflammation, steatosis, or fibrosis

Treatment prognosisStrict gluten-free diet (GFD)Glycemic control
(i) Can progress to fibrosis and cirrhosis(i) No progression to fibrosis or cirrhosis
(ii) Reversible with strict GFD(ii) Reversible with adequate glycemic control

Duration needed for complete resolution clinically, radiological, and histologically(i) Six months to one year(i) Depends on the achievement of adequate glycemic control but can be as early as four to five weeks
(ii) Reversibility is considered pathognomonic to celiac hepatitis