J Korean Diabetes Assoc.  2006 Jan;30(1):82-86.

A Case of Hepatic Glycogenosis in a Patient with Uncontrolled Type 1 Diabetes Mellitus

• Lee SH
• Kwon HS
• Shin JA
• Kim WC
• Kim JH
• Choi YH
• Yoon KH
• Cha BY
• Lee KW
• Son HY
• Kang SK

Abstract

When a patient with diabetes presents with hepatomegaly and increased level of liver enzymes, glycogenosis or nonalcoholic steatohepatitis (NASH) should be considered. Glycogenosis is mainly developed in patients with type 1 diabetes, when blood glucose level is poorly controlled, when a high dosage of insulin is administered in ketoacidosis, or when glucose is given to control hypoglycemia caused by high dosage of insulin. On the other hand, the main causes of NASH, which are known to mainly affect type 2 diabetes patients, are obesity, dyslipidemia or insulin resistance. Glycogenosis differs from NASH, the former being a reversible change that improves with the control of blood glucose level and the minimum dosage requirement of insulin, and the latter being a progressive disease that may lead to fibrosis or cirrhosis of the liver. However, clinical differentiation of the two diseases is difficult and liver biopsy is helpful for making a definite diagnosis. We present a type 1 diabetes patient with poorly controlled blood glucose level, who have had a frequent history of diabetic ketoacidosis, showing hepatomegaly and a slight increase in liver enzyme level. The patient was diagnosed as diabetic glycogenosis, confirmed by liver biopsy. Strict control of the blood glucose level resulted in rapid improvement showing the reversible nature of the disease.

Keyword

Hepatic glycogenosis; Hepatomegaly; Diabetes mellitus

MeSH Terms

Biopsy
Blood Glucose
Diabetes Mellitus
Diabetes Mellitus, Type 1*
Diabetic Ketoacidosis
Diagnosis
Dyslipidemias
Fatty Liver
Fibrosis
Glucose
Glycogen Storage Disease*
Hand
Hepatomegaly
Humans
Hypoglycemia
Insulin
Insulin Resistance
Ketosis
Liver
Obesity
Blood Glucose
Glucose
Insulin

Figure

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  Fig. 1 Abdominal X-ray on admission(A) and after 10 days of insulin treatment(B) showing regression of marked hepatomegaly after treatment.

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  Fig. 2 Abdominal CT on admission showing marked hepatomegaly

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  Fig. 3 Histology of liver biopsy. (A) swollen cytoplasm with intracytoplasmic vesicles and prominent cell border, forming a mosaic pattern (hematoxylin and eosin stain, ×400). (B) strongly positive for PAS stain suggesting cytoplasmic glycogenosis (×400).

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