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Mauriac syndrome

MedGen UID:
526123
•Concept ID:
C0221005
•
Disease or Syndrome
Synonym: Dwarfism-hepatomegaly-obesity-juvenile diabetes syndrome
SNOMED CT: Mauriac's syndrome (80660001); Dwarfism-hepatomegaly-obesity-juvenile diabetes syndrome (80660001); Mauriac syndrome (80660001)
 
Monarch Initiative: MONDO:0022435

Definition

A complication of poorly controlled type 1 diabetes mellitus in children characterized by linear growth impairment, glycogenic hepatopathy, and Cushingoid features. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Mauriac syndrome

Suggested Reading

PubMed

Discovery of a Genetic Metabolic Cause for Mauriac Syndrome in Type 1 Diabetes.
MacDonald MJ, Hasan NM, Ansari IU, Longacre MJ, Kendrick MA, Stoker SW
Diabetes 2016 Jul;65(7):2051-9. Epub 2016 Apr 5 doi: 10.2337/db16-0099. PMID: 27207549

Recent clinical studies

Etiology

Growth and development in type 1 diabetes.
Koren D
Curr Opin Endocrinol Diabetes Obes 2022 Feb 1;29(1):57-64. doi: 10.1097/MED.0000000000000694. PMID: 34864760
Glycogenic hepatopathy.
Khoury J, Zohar Y, Shehadeh N, Saadi T
Hepatobiliary Pancreat Dis Int 2018 Apr;17(2):113-118. Epub 2018 Feb 19 doi: 10.1016/j.hbpd.2018.02.006. PMID: 29709217
Growth in patients with type 1 diabetes.
Mitchell DM
Curr Opin Endocrinol Diabetes Obes 2017 Feb;24(1):67-72. doi: 10.1097/MED.0000000000000310. PMID: 27898589Free PMC Article
Mauriac's syndrome revisited.
Traisman HS, Traisman ES
Eur J Pediatr 1984 Sep;142(4):296-7. doi: 10.1007/BF00540257. PMID: 6541576
The Mauriac syndrome.
Mandell F, Berenberg W
Am J Dis Child 1974 Jun;127(6):900-2. doi: 10.1001/archpedi.1974.02110250126021. PMID: 4834782

Diagnosis

Severe Growth Failure and Poorly Controlled Type 1 Diabetes Mellitus in a 7-Year-Old Girl: Mauriac Syndrome.
Gillani S, Iqbal A, Kazmi NHS
J Coll Physicians Surg Pak 2019 Jun;29(6):590-591. doi: 10.29271/jcpsp.2019.06.590. PMID: 31133165
Glycogenic hepatopathy.
Khoury J, Zohar Y, Shehadeh N, Saadi T
Hepatobiliary Pancreat Dis Int 2018 Apr;17(2):113-118. Epub 2018 Feb 19 doi: 10.1016/j.hbpd.2018.02.006. PMID: 29709217
The Mauriac syndrome.
Morrison EY, McKenzie K
West Indian Med J 1989 Sep;38(3):180-2. PMID: 2694619
Mauriac's syndrome revisited.
Traisman HS, Traisman ES
Eur J Pediatr 1984 Sep;142(4):296-7. doi: 10.1007/BF00540257. PMID: 6541576
False positive sweat test, malnutrition, and the Mauriac syndrome.
Rosenfeld R, Spigelblatt L, Chicoine R
J Pediatr 1979 Feb;94(2):240-2. doi: 10.1016/s0022-3476(79)80832-x. PMID: 762615

Therapy

Glycogenic hepatopathy.
Khoury J, Zohar Y, Shehadeh N, Saadi T
Hepatobiliary Pancreat Dis Int 2018 Apr;17(2):113-118. Epub 2018 Feb 19 doi: 10.1016/j.hbpd.2018.02.006. PMID: 29709217
Mauriac syndrome still exists.
Franzese A, Iorio R, Buono P, Mascolo M, Mozzillo E, Valerio G
Diabetes Res Clin Pract 2001 Dec;54(3):219-21. doi: 10.1016/s0168-8227(01)00298-4. PMID: 11689279
The Mauriac syndrome.
Morrison EY, McKenzie K
West Indian Med J 1989 Sep;38(3):180-2. PMID: 2694619
The Mauriac syndrome.
Mandell F, Berenberg W
Am J Dis Child 1974 Jun;127(6):900-2. doi: 10.1001/archpedi.1974.02110250126021. PMID: 4834782
The Mauriac syndrome.
Najjar S, Ayash MA
Clin Pediatr (Phila) 1974 Sep;13(9):723-5. doi: 10.1177/000992287401300904. PMID: 4416595

Prognosis

Glycogenic hepatopathy.
Khoury J, Zohar Y, Shehadeh N, Saadi T
Hepatobiliary Pancreat Dis Int 2018 Apr;17(2):113-118. Epub 2018 Feb 19 doi: 10.1016/j.hbpd.2018.02.006. PMID: 29709217
Growth in patients with type 1 diabetes.
Mitchell DM
Curr Opin Endocrinol Diabetes Obes 2017 Feb;24(1):67-72. doi: 10.1097/MED.0000000000000310. PMID: 27898589Free PMC Article
Mauriac syndrome: growth failure and type 1 diabetes mellitus.
Kim MS, Quintos JB
Pediatr Endocrinol Rev 2008 Aug;5 Suppl 4:989-93. PMID: 18806715
Mauriac's syndrome revisited.
Traisman HS, Traisman ES
Eur J Pediatr 1984 Sep;142(4):296-7. doi: 10.1007/BF00540257. PMID: 6541576
The Mauriac syndrome.
Mandell F, Berenberg W
Am J Dis Child 1974 Jun;127(6):900-2. doi: 10.1001/archpedi.1974.02110250126021. PMID: 4834782

Clinical prediction guides

Glycogenic hepatopathy.
Khoury J, Zohar Y, Shehadeh N, Saadi T
Hepatobiliary Pancreat Dis Int 2018 Apr;17(2):113-118. Epub 2018 Feb 19 doi: 10.1016/j.hbpd.2018.02.006. PMID: 29709217
Clinical, Biochemical, and Histopathology Features of Patients With Glycogenic Hepatopathy.
Mukewar S, Sharma A, Lackore KA, Enders FT, Torbenson MS, Kamath PS, Roberts LR, Kudva YC
Clin Gastroenterol Hepatol 2017 Jun;15(6):927-933. Epub 2016 Dec 30 doi: 10.1016/j.cgh.2016.11.038. PMID: 28043933
Growth in patients with type 1 diabetes.
Mitchell DM
Curr Opin Endocrinol Diabetes Obes 2017 Feb;24(1):67-72. doi: 10.1097/MED.0000000000000310. PMID: 27898589Free PMC Article
Hepatopathy of Mauriac syndrome: a retrospective review from a tertiary liver centre.
Fitzpatrick E, Cotoi C, Quaglia A, Sakellariou S, Ford-Adams ME, Hadzic N
Arch Dis Child 2014 Apr;99(4):354-7. Epub 2014 Jan 10 doi: 10.1136/archdischild-2013-304426. PMID: 24412980
Periodontal findings in a patient with Mauriac syndrome: a case report.
Avula H
Spec Care Dentist 2012 Mar;32(2):66-9. doi: 10.1111/j.1754-4505.2012.00246.x. PMID: 22416989

Recent systematic reviews

Liver chemistries in glycogenic hepatopathy associated with type 1 diabetes mellitus: A systematic review and pooled analysis.
Haffar S, Izzy M, Habib H, Sugihara T, Li DK, Sharma A, Wang Z, Murad MH, Watt KD, Bazerbachi F
Liver Int 2021 Jul;41(7):1545-1555. Epub 2021 Feb 28 doi: 10.1111/liv.14827. PMID: 33595181

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