Reversible steatohepatosis in a young boy with brittle type 1 diabetes mellitus: mauriac syndrome

Shrivastava, Makardhwaj; Palkar, Atul; Padwal, Namita; Moulick, Nivedita

doi:10.1136/bcr.03.2011.3975

Cited by 7 publications

(5 citation statements)

References 1 publication

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“…6 Other authors have reported the same lack of liver synthetic dysfunction in MS-associated hepatopathy. 1,2,4,7,8 In contrast, our patient manifested with significantly elevated transaminases and abnormal liver synthetic function tests (prolonged PT, hypoalbuminemia). Although she was noted to have hepatomegaly for several years, her last set of liver transaminases was checked 4 years before her hospitalization.…”

Section: Discussioncontrasting

confidence: 54%

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Hepatomegaly and Liver Dysfunction in a 15-Year-Old Girl With Type 1 Diabetes Mellitus

Litao

El‐Baba

Buggs‐Saxton

2015

Clin Pediatr (Phila)

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Section: Discussioncontrasting

confidence: 54%

“…Adequate glycemic control can reverse not only the hepatomegaly but the other MS features of growth and pubertal retardation as well. 2,6,7,9,10…”

Section: Discussionmentioning

confidence: 99%

Hepatomegaly and Liver Dysfunction in a 15-Year-Old Girl With Type 1 Diabetes Mellitus

Litao

El‐Baba

Buggs‐Saxton

2015

Clin Pediatr (Phila)

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“…[ 1 ] Recently, there has been re-emergence of this syndrome, especially with the use of premix insulin. [ 2 ] Herein, we report such a case.…”

Section: Introductionmentioning

confidence: 99%

Re-emergence of a rare syndrome: A case of mauriac syndrome

Gutch¹,

Philip²,

Saran³

et al. 2013

Indian J Endocr Metab

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Mauriac syndrome is a rare syndrome associated with type 1 diabetes (T1DM) in children presenting with growth retardation, hepatomegaly, and cushingoid features. Recently, there has been re-emergence of this syndrome, especially with the use of premix insulin. A 15-year old type 1 diabetic boy, who was on premix insulin with erratic blood glucose, was referred to us for evaluation of short stature. He had significant short stature, hepatomegaly, and cushingoid features. His growth hormone (GH) stimulation was normal, and so was the overnight dexamethasone suppression test, based on which the diagnosis of Mauriac syndrome was reported. He was made to switch over to basal bolus regime, and was advised to follow-up for 6 months. He had reduction in hepatomegaly and a height gain of 3 cms.

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“…The etiology of Mauriac syndrome remains unclear; however, hypoinsulination and increased cortisolemia may play a role in increased glycogen storage [ 8 – 10 ]. Whether the pathomechanism is based on insulin deficiency or more on persisting hyperglycemia is still an important discussion and cannot be resolved in this patient.…”

Section: Discussionmentioning

confidence: 99%

Reversible severe glycogenic hepatopathy in type 1 diabetes

Laffolie

Kamrath

Burchert

et al. 2021

Wien Med Wochenschr

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Summary Case presentation We report a case of severe glycogenic hepatopathy in a 17-year-old boy with poorly controlled type 1 diabetes. On presentation, major findings included unexplained pronounced hepatomegaly and increased liver enzymes, ferritin, and triglycerides. Histology and electron microscopy evaluation showed severe glycogen storage, steatosis, and signs of fibrosis, resembling the histomorphological findings of Mauriac syndrome. After information about the nature of the disease and intensification of insulin therapy with insulin pump, liver enzymes, ferritin, and triglycerides normalized within 1 month. Conclusion Glycogenic hepatopathy is a rare but important potential complication in poorly controlled juvenile diabetic patients. With improved metabolic control, it is fully reversible.

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Reversible steatohepatosis in a young boy with brittle type 1 diabetes mellitus: mauriac syndrome

Cited by 7 publications

References 1 publication

Hepatomegaly and Liver Dysfunction in a 15-Year-Old Girl With Type 1 Diabetes Mellitus

Hepatomegaly and Liver Dysfunction in a 15-Year-Old Girl With Type 1 Diabetes Mellitus

Re-emergence of a rare syndrome: A case of mauriac syndrome

Reversible severe glycogenic hepatopathy in type 1 diabetes

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