Clinical, Biochemical, and Histopathology Features of Patients With Glycogenic Hepatopathy

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Abstract

Background & Aims Glycogenic hepatopathy, a syndrome characterized by hepatomegaly and increased liver transaminases in patients with type 1 diabetes, has not been well characterized in adults. We describe the clinical, biochemical, and histopathology profile of a cohort of patients with glycogenic hepatopathy. We also examined differences between patients with type 1 diabetes with versus without glycogenic hepatopathy. Methods We performed a case–control study of patients with type 1 diabetes diagnosed with glycogenic hepatopathy and patients with type 1 diabetes without glycogenic hepatopathy (control subjects). Cases were identified in the database of electronic medical records at Mayo Clinic, Rochester from January 1, 1998, through January 1, 2014. Age- and sex-matched control subjects were identified from a Mayo Clinic registry of patients with type 1 diabetes who had normal levels of liver enzymes. Demographic, clinical, laboratory, and histopathology data were collected and compared between cases and control subjects. The primary outcome was difference in frequency of diabetic ketoacidosis episodes and hemoglobin (Hb) A1c levels between cases and control subjects. Results Among the 36 patients diagnosed with glycogenic hepatopathy, 20 had undergone liver biopsy analysis. Most cases were female (n = 28; 77.8%). Abdominal pain was the most common symptom (n = 23; 63.9%); 28 patients (77.8%) had hepatomegaly. All patients had poor control of diabetes (mean HbA1c level, 11.2 ± 2.4%). A higher proportion of cases had recurrent episodes of diabetic ketoacidosis (61%) than control subjects (9%) (P =.009), and cases had a higher mean level of HbA1c (11.2 ± 2.4% vs 9.0 ± 2.2% in control subjects; P =.0004). Adult cases had higher levels of aspartate transaminase (312.5 IU/L; range, 245.5–775 IU/L) than pediatric cases (157; range, 104–267 IU/L; P =.02) and lower serum levels of albumin (3.7 ± 0.5 g/dL vs 4.3 ± 0.4 g/dL for pediatric cases; P =.008). Only 16.7% of pediatric patients with glycogenic hepatopathy had growth retardation. Levels of liver transaminases were normalized at follow-up examinations of 18 of 21 adult or pediatric patients with glycogenic hepatopathy. Conclusions More than half of patients with glycogenic hepatopathy and type 1 diabetes have recurrent episodes of diabetic ketoacidosis, and these patients have higher levels of HbA1c than patients with type 1 diabetes without glycogenic hepatopathy. We observed growth retardation in only about 17% of pediatric patients with glycogenic hepatopathy.

Original languageEnglish (US)
Pages (from-to)927-933
Number of pages7
JournalClinical Gastroenterology and Hepatology
Volume15
Issue number6
DOIs
StatePublished - Jun 1 2017

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Type 1 Diabetes Mellitus
Diabetic Ketoacidosis
Pediatrics
Hepatomegaly
Liver
Transaminases
Electronic Health Records
Growth
Aspartate Aminotransferases
Serum Albumin
Abdominal Pain
Registries
Hemoglobins
Demography
Databases
Biopsy
Enzymes

Keywords

  • AST
  • DKA
  • Glycogen Deposition
  • Mauriac Syndrome

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

Cite this

@article{c3cf68a505ce48c996e75ac4ea5f3199,
title = "Clinical, Biochemical, and Histopathology Features of Patients With Glycogenic Hepatopathy",
abstract = "Background & Aims Glycogenic hepatopathy, a syndrome characterized by hepatomegaly and increased liver transaminases in patients with type 1 diabetes, has not been well characterized in adults. We describe the clinical, biochemical, and histopathology profile of a cohort of patients with glycogenic hepatopathy. We also examined differences between patients with type 1 diabetes with versus without glycogenic hepatopathy. Methods We performed a case–control study of patients with type 1 diabetes diagnosed with glycogenic hepatopathy and patients with type 1 diabetes without glycogenic hepatopathy (control subjects). Cases were identified in the database of electronic medical records at Mayo Clinic, Rochester from January 1, 1998, through January 1, 2014. Age- and sex-matched control subjects were identified from a Mayo Clinic registry of patients with type 1 diabetes who had normal levels of liver enzymes. Demographic, clinical, laboratory, and histopathology data were collected and compared between cases and control subjects. The primary outcome was difference in frequency of diabetic ketoacidosis episodes and hemoglobin (Hb) A1c levels between cases and control subjects. Results Among the 36 patients diagnosed with glycogenic hepatopathy, 20 had undergone liver biopsy analysis. Most cases were female (n = 28; 77.8{\%}). Abdominal pain was the most common symptom (n = 23; 63.9{\%}); 28 patients (77.8{\%}) had hepatomegaly. All patients had poor control of diabetes (mean HbA1c level, 11.2 ± 2.4{\%}). A higher proportion of cases had recurrent episodes of diabetic ketoacidosis (61{\%}) than control subjects (9{\%}) (P =.009), and cases had a higher mean level of HbA1c (11.2 ± 2.4{\%} vs 9.0 ± 2.2{\%} in control subjects; P =.0004). Adult cases had higher levels of aspartate transaminase (312.5 IU/L; range, 245.5–775 IU/L) than pediatric cases (157; range, 104–267 IU/L; P =.02) and lower serum levels of albumin (3.7 ± 0.5 g/dL vs 4.3 ± 0.4 g/dL for pediatric cases; P =.008). Only 16.7{\%} of pediatric patients with glycogenic hepatopathy had growth retardation. Levels of liver transaminases were normalized at follow-up examinations of 18 of 21 adult or pediatric patients with glycogenic hepatopathy. Conclusions More than half of patients with glycogenic hepatopathy and type 1 diabetes have recurrent episodes of diabetic ketoacidosis, and these patients have higher levels of HbA1c than patients with type 1 diabetes without glycogenic hepatopathy. We observed growth retardation in only about 17{\%} of pediatric patients with glycogenic hepatopathy.",
keywords = "AST, DKA, Glycogen Deposition, Mauriac Syndrome",
author = "Saurabh Mukewar and Ayush Sharma and Lackore, {Kandace A.} and Enders, {Felicity T} and Michael Torbenson and Kamath, {Patrick Sequeira} and Roberts, {Lewis Rowland} and Kudva, {Yogish C}",
year = "2017",
month = "6",
day = "1",
doi = "10.1016/j.cgh.2016.11.038",
language = "English (US)",
volume = "15",
pages = "927--933",
journal = "Clinical Gastroenterology and Hepatology",
issn = "1542-3565",
publisher = "W.B. Saunders Ltd",
number = "6",

}

TY - JOUR

T1 - Clinical, Biochemical, and Histopathology Features of Patients With Glycogenic Hepatopathy

AU - Mukewar, Saurabh

AU - Sharma, Ayush

AU - Lackore, Kandace A.

AU - Enders, Felicity T

AU - Torbenson, Michael

AU - Kamath, Patrick Sequeira

AU - Roberts, Lewis Rowland

AU - Kudva, Yogish C

PY - 2017/6/1

Y1 - 2017/6/1

N2 - Background & Aims Glycogenic hepatopathy, a syndrome characterized by hepatomegaly and increased liver transaminases in patients with type 1 diabetes, has not been well characterized in adults. We describe the clinical, biochemical, and histopathology profile of a cohort of patients with glycogenic hepatopathy. We also examined differences between patients with type 1 diabetes with versus without glycogenic hepatopathy. Methods We performed a case–control study of patients with type 1 diabetes diagnosed with glycogenic hepatopathy and patients with type 1 diabetes without glycogenic hepatopathy (control subjects). Cases were identified in the database of electronic medical records at Mayo Clinic, Rochester from January 1, 1998, through January 1, 2014. Age- and sex-matched control subjects were identified from a Mayo Clinic registry of patients with type 1 diabetes who had normal levels of liver enzymes. Demographic, clinical, laboratory, and histopathology data were collected and compared between cases and control subjects. The primary outcome was difference in frequency of diabetic ketoacidosis episodes and hemoglobin (Hb) A1c levels between cases and control subjects. Results Among the 36 patients diagnosed with glycogenic hepatopathy, 20 had undergone liver biopsy analysis. Most cases were female (n = 28; 77.8%). Abdominal pain was the most common symptom (n = 23; 63.9%); 28 patients (77.8%) had hepatomegaly. All patients had poor control of diabetes (mean HbA1c level, 11.2 ± 2.4%). A higher proportion of cases had recurrent episodes of diabetic ketoacidosis (61%) than control subjects (9%) (P =.009), and cases had a higher mean level of HbA1c (11.2 ± 2.4% vs 9.0 ± 2.2% in control subjects; P =.0004). Adult cases had higher levels of aspartate transaminase (312.5 IU/L; range, 245.5–775 IU/L) than pediatric cases (157; range, 104–267 IU/L; P =.02) and lower serum levels of albumin (3.7 ± 0.5 g/dL vs 4.3 ± 0.4 g/dL for pediatric cases; P =.008). Only 16.7% of pediatric patients with glycogenic hepatopathy had growth retardation. Levels of liver transaminases were normalized at follow-up examinations of 18 of 21 adult or pediatric patients with glycogenic hepatopathy. Conclusions More than half of patients with glycogenic hepatopathy and type 1 diabetes have recurrent episodes of diabetic ketoacidosis, and these patients have higher levels of HbA1c than patients with type 1 diabetes without glycogenic hepatopathy. We observed growth retardation in only about 17% of pediatric patients with glycogenic hepatopathy.

AB - Background & Aims Glycogenic hepatopathy, a syndrome characterized by hepatomegaly and increased liver transaminases in patients with type 1 diabetes, has not been well characterized in adults. We describe the clinical, biochemical, and histopathology profile of a cohort of patients with glycogenic hepatopathy. We also examined differences between patients with type 1 diabetes with versus without glycogenic hepatopathy. Methods We performed a case–control study of patients with type 1 diabetes diagnosed with glycogenic hepatopathy and patients with type 1 diabetes without glycogenic hepatopathy (control subjects). Cases were identified in the database of electronic medical records at Mayo Clinic, Rochester from January 1, 1998, through January 1, 2014. Age- and sex-matched control subjects were identified from a Mayo Clinic registry of patients with type 1 diabetes who had normal levels of liver enzymes. Demographic, clinical, laboratory, and histopathology data were collected and compared between cases and control subjects. The primary outcome was difference in frequency of diabetic ketoacidosis episodes and hemoglobin (Hb) A1c levels between cases and control subjects. Results Among the 36 patients diagnosed with glycogenic hepatopathy, 20 had undergone liver biopsy analysis. Most cases were female (n = 28; 77.8%). Abdominal pain was the most common symptom (n = 23; 63.9%); 28 patients (77.8%) had hepatomegaly. All patients had poor control of diabetes (mean HbA1c level, 11.2 ± 2.4%). A higher proportion of cases had recurrent episodes of diabetic ketoacidosis (61%) than control subjects (9%) (P =.009), and cases had a higher mean level of HbA1c (11.2 ± 2.4% vs 9.0 ± 2.2% in control subjects; P =.0004). Adult cases had higher levels of aspartate transaminase (312.5 IU/L; range, 245.5–775 IU/L) than pediatric cases (157; range, 104–267 IU/L; P =.02) and lower serum levels of albumin (3.7 ± 0.5 g/dL vs 4.3 ± 0.4 g/dL for pediatric cases; P =.008). Only 16.7% of pediatric patients with glycogenic hepatopathy had growth retardation. Levels of liver transaminases were normalized at follow-up examinations of 18 of 21 adult or pediatric patients with glycogenic hepatopathy. Conclusions More than half of patients with glycogenic hepatopathy and type 1 diabetes have recurrent episodes of diabetic ketoacidosis, and these patients have higher levels of HbA1c than patients with type 1 diabetes without glycogenic hepatopathy. We observed growth retardation in only about 17% of pediatric patients with glycogenic hepatopathy.

KW - AST

KW - DKA

KW - Glycogen Deposition

KW - Mauriac Syndrome

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U2 - 10.1016/j.cgh.2016.11.038

DO - 10.1016/j.cgh.2016.11.038

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JO - Clinical Gastroenterology and Hepatology

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SN - 1542-3565

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