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Hepatopathy of Mauriac syndrome: a retrospective review from a tertiary liver centre

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Hepatopathy of Mauriac syndrome : a retrospective review from a tertiary liver centre. / Fitzpatrick, Emer; Cotoi, Corina ; Quaglia, Alberto; Sakellariou, S; Ford-Adams, Martha; Hadzic, Nedim.

In: Archives of Disease in Childhood, Vol. 99, No. 4, 04.2014, p. 354-357.

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Harvard

Fitzpatrick, E, Cotoi, C, Quaglia, A, Sakellariou, S, Ford-Adams, M & Hadzic, N 2014, 'Hepatopathy of Mauriac syndrome: a retrospective review from a tertiary liver centre', Archives of Disease in Childhood, vol. 99, no. 4, pp. 354-357. https://doi.org/10.1136/archdischild-2013-304426

APA

Fitzpatrick, E., Cotoi, C., Quaglia, A., Sakellariou, S., Ford-Adams, M., & Hadzic, N. (2014). Hepatopathy of Mauriac syndrome: a retrospective review from a tertiary liver centre. Archives of Disease in Childhood, 99(4), 354-357. https://doi.org/10.1136/archdischild-2013-304426

Vancouver

Fitzpatrick E, Cotoi C, Quaglia A, Sakellariou S, Ford-Adams M, Hadzic N. Hepatopathy of Mauriac syndrome: a retrospective review from a tertiary liver centre. Archives of Disease in Childhood. 2014 Apr;99(4):354-357. https://doi.org/10.1136/archdischild-2013-304426

Author

Fitzpatrick, Emer ; Cotoi, Corina ; Quaglia, Alberto ; Sakellariou, S ; Ford-Adams, Martha ; Hadzic, Nedim. / Hepatopathy of Mauriac syndrome : a retrospective review from a tertiary liver centre. In: Archives of Disease in Childhood. 2014 ; Vol. 99, No. 4. pp. 354-357.

Bibtex Download

@article{f59161199e454bc8af13f868847f48c7,
title = "Hepatopathy of Mauriac syndrome: a retrospective review from a tertiary liver centre",
abstract = "Background: Mauriac syndrome is characterised by growth failure, cushingoid appearance and hepatomegaly which occurs in patients with insulin dependent diabetes and was first described shortly after the introduction of insulin as a treatment for the condition.Objective: To describe the clinical features, histological findings and outcome of young people with glycogenic hepatopathy, the hepatic manifestation of Mauriac syndrome (MS).Design: Retrospective cohort study.Patients: Young people with glycogenic hepatopathy.Setting: Tertiary paediatric hepatology unit.Results: Thirty-one young people (16 M), median age of 15.1 years (IQR 14–16.2) presented within the study period. Median age of diagnosis of diabetes was 10 years (IQR 8–11). Median insulin requirement was 1.33 units/kg/day; median HbA1c was 96.7 mmol/mol (IQR 84.7–112.0). Growth was impaired: median height z-score was −1.01 (−1.73 to 0.4) and median body mass index (BMI) z-score was 0.28 (−0.12 to 0.67). Hepatomegaly was universal with splenomegaly in 16{\%}. Transaminases were abnormal with a median aspartate aminotransferase (AST) of 76 IU/L and gamma glutamyltransferase of 71 IU/L. Liver biopsy was undertaken in 19 (61{\%}). All showed enlarged hepatocytes with clear cytoplasm with glycogenated nuclei in 17. Steatosis was present in the majority. Inflammation was present in 8 (42{\%}). Fibrosis was seen in 14 (73{\%}) and was generally mild though 2 had bridging fibrosis. Megamitochondria were described in 7. Presence of megamitochondria correlated with AST elevation (p=0.026) and fibrosis on biopsy (p=0.007). At follow-up 17 children had normal or improved transaminases, in 13 there was no change. Transaminases followed the trend of the child's HbA1c.Conclusions: Despite modern insulin regimens and monitoring in children with type 1 diabetes, MS still exists. Significant steatosis, inflammation and fibrosis were all seen in liver biopsies.",
author = "Emer Fitzpatrick and Corina Cotoi and Alberto Quaglia and S Sakellariou and Martha Ford-Adams and Nedim Hadzic",
year = "2014",
month = "4",
doi = "10.1136/archdischild-2013-304426",
language = "English",
volume = "99",
pages = "354--357",
journal = "Archives of Disease in Childhood",
issn = "0003-9888",
publisher = "BMJ Publishing Group Ltd",
number = "4",

}

RIS (suitable for import to EndNote) Download

TY - JOUR

T1 - Hepatopathy of Mauriac syndrome

T2 - a retrospective review from a tertiary liver centre

AU - Fitzpatrick, Emer

AU - Cotoi, Corina

AU - Quaglia, Alberto

AU - Sakellariou, S

AU - Ford-Adams, Martha

AU - Hadzic, Nedim

PY - 2014/4

Y1 - 2014/4

N2 - Background: Mauriac syndrome is characterised by growth failure, cushingoid appearance and hepatomegaly which occurs in patients with insulin dependent diabetes and was first described shortly after the introduction of insulin as a treatment for the condition.Objective: To describe the clinical features, histological findings and outcome of young people with glycogenic hepatopathy, the hepatic manifestation of Mauriac syndrome (MS).Design: Retrospective cohort study.Patients: Young people with glycogenic hepatopathy.Setting: Tertiary paediatric hepatology unit.Results: Thirty-one young people (16 M), median age of 15.1 years (IQR 14–16.2) presented within the study period. Median age of diagnosis of diabetes was 10 years (IQR 8–11). Median insulin requirement was 1.33 units/kg/day; median HbA1c was 96.7 mmol/mol (IQR 84.7–112.0). Growth was impaired: median height z-score was −1.01 (−1.73 to 0.4) and median body mass index (BMI) z-score was 0.28 (−0.12 to 0.67). Hepatomegaly was universal with splenomegaly in 16%. Transaminases were abnormal with a median aspartate aminotransferase (AST) of 76 IU/L and gamma glutamyltransferase of 71 IU/L. Liver biopsy was undertaken in 19 (61%). All showed enlarged hepatocytes with clear cytoplasm with glycogenated nuclei in 17. Steatosis was present in the majority. Inflammation was present in 8 (42%). Fibrosis was seen in 14 (73%) and was generally mild though 2 had bridging fibrosis. Megamitochondria were described in 7. Presence of megamitochondria correlated with AST elevation (p=0.026) and fibrosis on biopsy (p=0.007). At follow-up 17 children had normal or improved transaminases, in 13 there was no change. Transaminases followed the trend of the child's HbA1c.Conclusions: Despite modern insulin regimens and monitoring in children with type 1 diabetes, MS still exists. Significant steatosis, inflammation and fibrosis were all seen in liver biopsies.

AB - Background: Mauriac syndrome is characterised by growth failure, cushingoid appearance and hepatomegaly which occurs in patients with insulin dependent diabetes and was first described shortly after the introduction of insulin as a treatment for the condition.Objective: To describe the clinical features, histological findings and outcome of young people with glycogenic hepatopathy, the hepatic manifestation of Mauriac syndrome (MS).Design: Retrospective cohort study.Patients: Young people with glycogenic hepatopathy.Setting: Tertiary paediatric hepatology unit.Results: Thirty-one young people (16 M), median age of 15.1 years (IQR 14–16.2) presented within the study period. Median age of diagnosis of diabetes was 10 years (IQR 8–11). Median insulin requirement was 1.33 units/kg/day; median HbA1c was 96.7 mmol/mol (IQR 84.7–112.0). Growth was impaired: median height z-score was −1.01 (−1.73 to 0.4) and median body mass index (BMI) z-score was 0.28 (−0.12 to 0.67). Hepatomegaly was universal with splenomegaly in 16%. Transaminases were abnormal with a median aspartate aminotransferase (AST) of 76 IU/L and gamma glutamyltransferase of 71 IU/L. Liver biopsy was undertaken in 19 (61%). All showed enlarged hepatocytes with clear cytoplasm with glycogenated nuclei in 17. Steatosis was present in the majority. Inflammation was present in 8 (42%). Fibrosis was seen in 14 (73%) and was generally mild though 2 had bridging fibrosis. Megamitochondria were described in 7. Presence of megamitochondria correlated with AST elevation (p=0.026) and fibrosis on biopsy (p=0.007). At follow-up 17 children had normal or improved transaminases, in 13 there was no change. Transaminases followed the trend of the child's HbA1c.Conclusions: Despite modern insulin regimens and monitoring in children with type 1 diabetes, MS still exists. Significant steatosis, inflammation and fibrosis were all seen in liver biopsies.

U2 - 10.1136/archdischild-2013-304426

DO - 10.1136/archdischild-2013-304426

M3 - Article

VL - 99

SP - 354

EP - 357

JO - Archives of Disease in Childhood

JF - Archives of Disease in Childhood

SN - 0003-9888

IS - 4

ER -

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