Mauriac Syndrome Still Exists in Poorly Controlled Type 1 Diabetes: A Report of Two Cases and Literature Review

Alhajjaj, Alya Hassan; Aljishi, Fatemah K

doi:10.7759/cureus.14704

Cited by 9 publications

(10 citation statements)

References 6 publications

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“…Newer insulin formulations enabled better round-theclock insulin delivery and glycemic control, leading to a dramatic decrease in poorly controlled diabetics, at least in the developed world, thus almost eliminating the syndrome. However, reports of its cardinal features among young people with newly diagnosed type 1 DM still appear in the medical literature [12,13]. Although the exact 5 etiopathogenetic mechanism has not been fully studied, it is believed that fluctuations in blood glucose values with subsequent alterations in insulin production and delivery lead to increased excessive glycogen storage within the liver.…”

Section: Discussionmentioning

confidence: 99%

Glycogenic Hepatopathy in a Teenage Girl with Diabetes Mellitus Type 1

Kintrilis¹

2023

Series Endo Diab Met.

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Glycogenic hepatopathy (GH) refers to a relatively rare complication of diabetes mellitus (DM) type 1 which constitutes of reversible accumulation of excess hepatic glycogen, usually manifesting in the form of liver enzyme elevation along with hepatomegaly. The occurrence of the disorder is most commonly related to inadequate control of blood sugars. Herein, we report upon a case of an 18-year-old girl presenting with severe transaminase elevations owing to poor metabolic control, as well as the progression of her enzymes and general condition during and after her hospitalization.

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Section: Discussionmentioning

confidence: 99%

Glycogenic Hepatopathy in a Teenage Girl with Diabetes Mellitus Type 1

Kintrilis¹

2023

Series Endo Diab Met.

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“…In this case, a liver biopsy to differentiate these two entities is recommended. 17,18 Hypoxic hepatitis is found in approximately 1-2% of adults hospitalized in Intensive Care Units and is associated with approximately 50% of in-hospital mortality. 10,11 Management of hypoxic hepatitis involves quick diagnosis and treatment of underlying conditions and comorbidities.…”

Section: Discussionmentioning

confidence: 99%

“…10,11 Hypoxic hepatitis is not included in the official list of complications of diabetic ketoacidosis, and the statistics of its incidence in children are unknown. 18,19 Therefore, awareness of this complication, early detection by monitoring aminotransferases, LDH, liver function parameters, and early treatment of underlying diseases is essential for the prognosis of these patients. 6,18…”

Section: Discussionmentioning

confidence: 99%

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Hypoxic hepatitis as a complication of newly diagnosed type 1 diabetes in a teenager

Buczkowski¹,

Oóg-Zabolska²,

Gulczyski³

et al. 2022

Autops Case Rep

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Hypoxic hepatitis is a rare complication of type 1 diabetes with unknown prevalence in Pediatrics. We present a case report of an 11-year-old boy admitted to the ER in the spring of 2020 (the beginning of the COVID19 pandemic in Poland) due to nausea, abdominal pain, and weight loss. A diagnosis of type 1 diabetes accompanied by severe ketoacidosis (pH 6.9, blood glucose 632mg/dl, ketone bodies in urine -150mg/dl) was made. The hyperglycemia, ketoacidosis, and waterelectrolyte disturbances were treated in the Pediatric Intensive Care Unit. On day 4, the boy developed fulminant septic shock with high aminotransferases (AST 9026 U/l, ALT 3559 U/l). CT scan revealed hepatic enlargement and steatosis. Acute viral hepatitis was suspected. The levels of anti-CMV IgM and IgG antibodies were slightly elevated. At autopsy, the liver was enlarged, with petechial bleedings on the surface. The liver parenchyma was congested, with signs of steatosis. Microscopically, there was extensive centrilobular necrosis, acute passive sinusoidal congestion, and steatosis of hepatocytes. There were no signs of CMV infection. Based on the entire clinicopathological picture, the patient was diagnosed with hypoxic hepatitis, complicated by septic shock and multiple organ failure.

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“…Dysglycemia can be due to several reasons: non‐physiologic treatment plan, unaffordable insulin or expensive insulin analogs which are unaffordable (hence rationed or omitted), and/or with inadequate or no SMBG. Mauriac syndrome, 88 due to very severe longstanding dysglycemia, is characterized by growth failure, hepatomegaly with glycogenic hepatopathy, steatosis, and late pubertal development. Most examples of Mauriac Syndrome also have frequent significant psychosocial ramifications, family conflicts, and major problems with insulin and monitoring.…”

Section: Complications and Screeningmentioning

confidence: 99%

ISPAD Clinical Practice Consensus Guidelines 2022: Management of the child, adolescent, and young adult with diabetes in limited resource settings

Virmani

Brink²,

Middlehurst

et al. 2022

Pediatric Diabetes

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This guideline provides updated and consolidated guidance on best-possible care to children, adolescents, and young adults with type 1 diabetes (T1D) and type 2 diabetes (T2D) in widely varying situations when human and medical resources are acutely or chronically limited for any reason.The management of T1D should be as physiological as possible even in limited resource settings (LRS), to improve care and decrease morbidity and mortality. These recommendations are not aimed at endorsing suboptimal care, but at improving care by making the best possible use of available resources, while constantly endeavoring to reach the next level.

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Mauriac Syndrome Still Exists in Poorly Controlled Type 1 Diabetes: A Report of Two Cases and Literature Review

Cited by 9 publications

References 6 publications

Glycogenic Hepatopathy in a Teenage Girl with Diabetes Mellitus Type 1

Glycogenic Hepatopathy in a Teenage Girl with Diabetes Mellitus Type 1

Hypoxic hepatitis as a complication of newly diagnosed type 1 diabetes in a teenager

ISPAD Clinical Practice Consensus Guidelines 2022: Management of the child, adolescent, and young adult with diabetes in limited resource settings

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