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

Gutch, Manish; Philip, Rajeev; Saran, Sonal; Tyagi, Rajiv; Gupta, Keshav Kumar

doi:10.4103/2230-8210.119611

Cited by 20 publications

(18 citation statements)

References 6 publications

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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%

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%

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

Kočova¹,

Milenkova²

2018

SAGE Open Medical Case Reports

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“…Based on published reports in English literature, approximately 98% of GH cases were reported in T1DM, while the remaining 2% is caused by T2DM. The incidence of this syndrome has decreased significantly with the introduction of long-acting insulin and better control of blood sugar, but unfortunately, it still exists[ 52 ].…”

Section: Incidencementioning

confidence: 99%

“…In patients with a classic presentation of Mauriac syndrome, the wide fluctuations between hyper- and hypoglycemia-a pattern suggestive of under- and over-insulinization with secondary hyperadrenalism-possibly caused the cushingoid features. The pathogenesis of growth retardation in those initial cases reported in children was thought to be multifactorial[ 52 ].…”

Section: Pathogenesismentioning

confidence: 99%

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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“…One of the characteristics of the Mauriac syndrome is obesity, however, recently some non-obese patients have been reported as well [31].…”

Section: Discussionmentioning

confidence: 99%