open access

Vol 6, No 3 (2017)
REVIEW ARTICLES
Published online: 2017-09-29
Get Citation

Mauriac syndrome — is already a history?

Ewa Otto-Buczkowska, Natalia Jainta
DOI: 10.5603/DK.2017.0016
·
Clinical Diabetology 2017;6(3):101-104.

open access

Vol 6, No 3 (2017)
REVIEW ARTICLES
Published online: 2017-09-29

Abstract

In 1930 Mauriac described a syndrome characterized by the presence of growth impairment, hepatomegaly, delayed puberty and cushingoid features in patients with poorly controlled type 1 diabetes mellitus (T1DM). At that time, Mauriac syndrome was a common occur­rence. This state was maintained even in the fifties and sixties of the last century. The introduction of modern methods of insulin and glucose monitoring decisively reduced the frequency of occurrence of this syndrome. The incidence of Mauriac syndrome decreased dramati­cally, although it is still being reported. Often in these cases the patients present only some symptoms of this syndrome. The introduction of diagnostic tests allowed to find the genetic basis of these disorders in some of the cases. (Clin Diabetol 2017; 6, 3: 101–104)

Abstract

In 1930 Mauriac described a syndrome characterized by the presence of growth impairment, hepatomegaly, delayed puberty and cushingoid features in patients with poorly controlled type 1 diabetes mellitus (T1DM). At that time, Mauriac syndrome was a common occur­rence. This state was maintained even in the fifties and sixties of the last century. The introduction of modern methods of insulin and glucose monitoring decisively reduced the frequency of occurrence of this syndrome. The incidence of Mauriac syndrome decreased dramati­cally, although it is still being reported. Often in these cases the patients present only some symptoms of this syndrome. The introduction of diagnostic tests allowed to find the genetic basis of these disorders in some of the cases. (Clin Diabetol 2017; 6, 3: 101–104)

Get Citation

Keywords

Mauriac syndrome; growth retardation; poorly controlled diabetes

About this article
Title

Mauriac syndrome — is already a history?

Journal

Clinical Diabetology

Issue

Vol 6, No 3 (2017)

Pages

101-104

Published online

2017-09-29

DOI

10.5603/DK.2017.0016

Bibliographic record

Clinical Diabetology 2017;6(3):101-104.

Keywords

Mauriac syndrome
growth retardation
poorly controlled diabetes

Authors

Ewa Otto-Buczkowska
Natalia Jainta

References (31)
  1. Mauriac P. Gros ventre, hepatomegalie, troubles de croissance chez les enfants diabetiques traites depuis plusiers annee par l’insuline. Gaz Hebd Med Bordeaux. 1930; 26: 402–410.
  2. Mandell F, Berenberg W. The Mauriac syndrome. Am J Dis Child. 1974; 127(6): 900–902.
  3. Najjar S, Ayash MA. The Mauriac syndrome. Clin Pediatr (Phila). 1974; 13(9): 723–725.
  4. Olszowska L, Ludwiczak H. [Mauriac syndrome--an ever present problem in infantile diabetes]. Pediatr Pol. 1976; 51(8): 931–935.
  5. Otto-Buczkowska E, Rzepka J, Sońta-Jakimczyk D, et al. [Somatic development of diabetic children]. Pediatr Pol. 1973; 48(3): 307–315.
  6. Bognetti E, Riva MC, Bonfanti R, et al. Growth changes in children and adolescents with short-term diabetes. Diabetes Care. 1998; 21(8): 1226–1229.
  7. Holl RW, Heinze E, Seifert M, et al. Longitudinal analysis of somatic development in paediatric patients with IDDM: genetic influences on height and weight. Diabetologia. 1994; 37(9): 925–929.
  8. Jackson R. Growth and Maturation of Children with Insulin-Dependent Diabetes Mellitus. Pediatric Clinics of North America. 1984; 31(3): 545–567.
  9. Jos J, Méteyer I, Farkas D, et al. [Growth of children with insulin-dependent diabetes. Study of 104 cases]. Arch Pediatr. 1996; 3(3): 218–226.
  10. Morrison EY, McKenzie K. The Mauriac syndrome. West Indian Med J. 1989; 38(3): 180–182.
  11. Madhu SV, Jain R, Kant S, et al. Mauriac syndrome: A rare complication of type 1 diabetes mellitus. Indian J Endocrinol Metab. 2013; 17(4): 764–765.
  12. Oeschgef VV, Prieto M. [Mauriac syndrome in Argentina in the XXI century: series of 5 cases]. Arch Argent Pediatr. 2014; 112(2): e46–e49.
  13. Dias J, Martins S, Carvalho S, et al. Mauriac syndrome still exists. Endocrinol Nutr. 2013; 60(5): 245–248.
  14. Schmetz AVN, Dekker-Maas MH, den Breejen MP, et al. [Mauriac syndrome--a rare complication of type 1 diabetes mellitus]. Ned Tijdschr Geneeskd. 2012; 156(28): A4678.
  15. Kim MS, Quintos JB. Mauriac syndrome: growth failure and type 1 diabetes mellitus. Pediatr Endocrinol Rev. 2008; 5 Suppl 4: 989–993.
  16. Otto-Buczkowska E, Smigla K, Sliwa F. [Microscopic liver pattern in diabetic children]. Wiad Lek. 1974; 27(24): 2111–2114.
  17. Fitzpatrick E, Cotoi C, Quaglia A, et al. Hepatopathy of Mauriac syndrome: a retrospective review from a tertiary liver centre. Arch Dis Child. 2014; 99(4): 354–357.
  18. Elder CJ, Natarajan A. Mauriac syndrome--a modern reality. J Pediatr Endocrinol Metab. 2010; 23(3): 311–313.
  19. Mitchell DM. Growth in patients with type 1 diabetes. Curr Opin Endocrinol Diabetes Obes. 2017; 24(1): 67–72.
  20. Al Sarkhy AA, Zaidi ZA, Babiker AM. Glycogenic hepatopathy, an underdiagnosed cause of relapsing hepatitis in uncontrolled type 1 diabetes mellitus. Saudi Med J. 2017; 38(1): 89–92.
  21. Atmaca M, Ucler R, Kartal M, et al. Glycogenic Hepatopathy in Type 1 Diabetes Mellitus. Case Reports Hepatol. 2015; 2015: 236143.
  22. Giordano S, Martocchia A, Toussan L, et al. Diagnosis of hepatic glycogenosis in poorly controlled type 1 diabetes mellitus. World J Diabetes. 2014; 5(6): 882–888.
  23. Hernández-Quiles C, Fernández-Ojeda MR, Solanilla Rodríguez R, et al. [Mauriac syndrome: a liver disease that differs from steatosis of diabetes]. Rev Clin Esp (Barc). 2013; 213(3): 169–170.
  24. Brouwers MC, Ham JC, Wisse E, et al. Elevated lactate levels in patients with poorly regulated type 1 diabetes and glycogenic hepatopathy: a new feature of Mauriac syndrome. Diabetes Care. 2015; 38(2): e11–e12.
  25. Deemer KS, Alvarez GF. A Rare Case of Persistent Lactic Acidosis in the ICU: Glycogenic Hepatopathy and Mauriac Syndrome. Case Rep Crit Care. 2016; 2016: 6072909.
  26. Jung InAh, Cho WK, Jeon YJ, et al. Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome. Korean J Pediatr. 2015; 58(6): 234–237.
  27. Nagesh VS, Kalra S. Type 1 diabetes: Syndromes in resource-challenged settings. J Pak Med Assoc. 2015; 65(6): 681–685.
  28. MacDonald MJ, Hasan NM, Ansari IUH, et al. Discovery of a Genetic Metabolic Cause for Mauriac Syndrome in Type 1 Diabetes. Diabetes. 2016; 65(7): 2051–2059.
  29. Chai-udom R, Sahakitrungruang T, Wacharasindhu S, et al. A girl with permanent neonatal diabetes due to KCNJ11 mutation presented with Mauriac syndrome after improper adjustment in sulfonylurea dosage over 6 years. Journal of Pediatric Endocrinology and Metabolism. 2016; 29(9).
  30. Chowdhury S. Puberty and type 1 diabetes. Indian Journal of Endocrinology and Metabolism. 2015; 19(7): 51.
  31. Gutch M, Philip R, Saran S, et al. Re-emergence of a rare syndrome: A case of mauriac syndrome. Indian J Endocrinol Metab. 2013; 17(Suppl 1): S283–S285.

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

 

Wydawcą serwisu jest  "Via Medica sp. z o.o." sp.k., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail:  viamedica@viamedica.pl