Mauriac syndrome
| Classification according to ICD-10 | |
|---|---|
| E10.7 | Primarily insulin-dependent diabetes mellitus [type 1 diabetes] with multiple complications |
| ICD-10 online (WHO version 2019) | |
The Mauriac syndrome refers to a diabetic dwarfism and is considered a rare complication of diabetes type I.
Synonym: Infantile diabetes with glycogenosis
The name refers to the first author of the first description from 1930 by the French doctor Pierre Mauriac (1882–1963).
Clinical manifestations
Clinical criteria are:
- Juvenile diabetes mellitus, difficult to adjust due to initial counterregulations
- Chronic ketonuria
- Pronounced hepatomegaly with glycogen storage without splenomegaly
- Short stature with delayed ossification and sexual maturity
- Tribe obesity with a full moon face
- Hyperlipidemia
- osteoporosis
Old insulin preparations that are no longer in use are the cause .
literature
- CJ Elder, A. Natarajan: Mauriac syndrome - a modern reality. In: Journal of pediatric endocrinology & metabolism: JPEM. Vol. 23, No. 3, March 2010, pp. 311-313, PMID 20480733 .
Individual evidence
- ↑ a b Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
- ↑ P. Mauriac .: Gros ventre, hépatomégalie, troubles de la croissance chez les enfants diabétiques traités depuis plusieurs années par l'insuline. In: Gazette Sciences médicales (Bordeaux) Vol. 51, 1930, p. 402
- ↑ A. Windorfer The Mauriac Syndrome. Childhood diabetes with secondary glycogenosis
