Mauriac Syndrome in the Age of Newer Insulin Analogues

Ch, Venkatesh; rasekaran,; Baineni, Revanth; Srinivasaraghavan, Rangan; Bhat, Vishnu

doi:10.21767/2471-805x.100003

Cited by 3 publications

(3 citation statements)

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“…Isolated patients with this syndrome are still being reported. 3 , 7 – 10 The pathophysiology of this entity has not been elucidated. High index of suspicion for this syndrome should be maintained in a poorly controlled diabetic child with delayed growth.…”

Section: Discussionmentioning

confidence: 99%

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Old syndrome–new approach: Mauriac syndrome treated with continuous insulin delivery

Kočova¹,

Milenkova²

2018

SAGE Open Medical Case Reports

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Mauriac syndrome has rarely been reported in children and adolescents with a poorly controlled diabetes mellitus type 1. However, it still occurs despite the worldwide improvements of metabolic control. The risks have not been elucidated. We present a 13.5-year-old boy with a typical clinical presentation of Mauriac syndrome consisting of growth delay, cushingoid appearance, hepatomegaly, and delayed puberty. A stepwise correction of glycemic control was introduced using continuous insulin delivery. All symptoms improved during the 2.5-year follow-up. No retinopathy occurred. This patient with Mauriac syndrome followed with continuous glucose monitoring and treated with continuous insulin delivery, resulting in no retinopathy after 2.5 years of follow-up. We suggest that this approach should be recommended in patients with Mauriac syndrome.

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Section: Discussionmentioning

confidence: 99%

“…Improvement of the metabolic control in children with Mauriac syndrome is mandatory and includes treatment with insulin analogues; 8 however, if it is introduced too fast, retinopathy can be induced or existing retinopathy and/or nephropathy can be aggravated within months. 5 , 6…”

Section: Discussionmentioning

confidence: 99%

Old syndrome–new approach: Mauriac syndrome treated with continuous insulin delivery

Kočova¹,

Milenkova²

2018

SAGE Open Medical Case Reports

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“…Despite the recent advances in pathology and imaging, glycogen hepatopathy is only diagnosed by liver biopsy [5,6]. It is worth mentioning that synthetic liver functions are not affected in glycogen hepatopathy which could narrow the differential diagnosis [7]. A recent small case series found that elevated levels of lactic acid appeared to be a part of the disease process [8].…”

Section: Discussionmentioning

confidence: 99%

Acute Benign Hepatitis Due to Glycogen Hepatopathy

Ekladious¹

2021

Jap J Clin & Med Res

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Glycogen hepatopathy is a very rare and forgotten complication of poorly controlled type 1 diabetes mellitus. Glycogen hepatopathy may also present in type II diabetes mellitus, especially when managed with high doses of insulin. Although it is a benign condition, it is rarely diagnosed in a timely manner. It is characterised by hepatomegaly causing abdominal pain due to stretch on the liver capsule causing capsulitis, and derangement of liver enzymes. In this article we report a patient who presented with severe abdominal pain, hepatomegaly and transaminitis, the symptoms persisted for one year before the diagnosis of glycogen hepatopathy was made.

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Glycogenic hepatopathy: A narrative review

Sherigar

Castro

Yin

et al. 2018

WJH

112

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Glycogenic hepatopathy (GH) is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver dysfunction with elevated liver enzymes and associated hepatomegaly caused by the reversible accumulation of excess glycogen in the hepatocytes. It is predominantly seen in patients with longstanding type 1 diabetes mellitus and rarely reported in association with type 2 diabetes mellitus. Although it was first observed in the pediatric population, since then, it has been reported in adolescents and adults with or without ketoacidosis. The association of GH with hyperglycemia in diabetes has not been well established. One of the essential elements in the pathophysiology of development of GH is the wide fluctuation in both glucose and insulin levels. GH and non-alcoholic fatty liver disease (NAFLD) are clinically indistinguishable, and latter is more prevalent in diabetic patients and can progress to advanced liver disease and cirrhosis. Gradient dual-echo MRI can distinguish GH from NAFLD; however, GH can reliably be diagnosed only by liver biopsy. Adequate glycemic control can result in complete remission of clinical, laboratory and histological abnormalities. There has been a recent report of varying degree of liver fibrosis identified in patients with GH. Future studies are required to understand the biochemical defects underlying GH, noninvasive, rapid diagnostic tests for GH, and to assess the consequence of the fibrosis identified as severe fibrosis may progress to cirrhosis. Awareness of this entity in the medical community including specialists is low. Here we briefly reviewed the English literature on pathogenesis involved, recent progress in the evaluation, differential diagnosis, and management.

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Mauriac Syndrome in the Age of Newer Insulin Analogues

Cited by 3 publications

References 1 publication

Old syndrome–new approach: Mauriac syndrome treated with continuous insulin delivery

Old syndrome–new approach: Mauriac syndrome treated with continuous insulin delivery

Acute Benign Hepatitis Due to Glycogen Hepatopathy

Glycogenic hepatopathy: A narrative review

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