Achard-Thiers Syndrome
| Classification according to ICD-10 | |
|---|---|
| E25.9 | Adrenogenital disorder, unspecified |
| ICD-10 online (WHO version 2019) | |
The Achard-Thiers syndrome , formerly known as diabetes bearded women called, is a rare special form of diabetes mellitus . It is one of the rare endocrinological complex diseases in which diabetes mellitus is present and is a special form of Cushing's syndrome I that only occurs in women .
etiology
The cause of the endocrine disorder is usually a basophilic pituitary adenoma or an adrenal cortex tumor ( adrenal cortex tumor). It mainly affects postmenopausal women .
Symptoms
The symptoms of Achard-Thiers syndrome are mainly caused by the overproduction of male sex hormones ( hyperandrogenemia ). These are hirsutism (including “lady's beard”), obesity , diabetes mellitus, hair loss (alopecia), high blood pressure ( arterial hypertension ), clitoral hypertrophy and missed menstruation ( amenorrhea ).
therapy
Both the pituitary adenomas and the adrenal gland tumors are usually removed surgically. In the case of adrenalectomy , this procedure is called adrenalectomy . After the operation, a lifelong intake of hormone substitutes is necessary to prevent adrenal insufficiency (Addison's disease).
Initial description
Achard-Thiers syndrome was first described in 1921 by the French internist Émile Charles Achard (1860–1944) and the French clinician Joseph Thiers (1885–1960) as “diabetes of bearded women” ( diabète des femmes à barbe ). They recognized for the first time the connection between an excessively high male hormone level (hyperandrogenemia) and a disorder of the sugar metabolism.
literature
- W. Malaisse et al: Diabetes in bearded women (Achard-Thiers syndrome). In: Diabetologia 1, 1966, pp. 155-161. doi : 10.1007 / BF01257906
- L. Adamska: Achard-Thiers Syndrome. In: Fudahr Pol 13, 1962, pp. 413-423.
- I. Lubowe: Achard-Thiers syndrome. In: Arch Dermatol 103, 1971, pp. 544-545. PMID 5580302
Web links
- Cushing's Syndrome (theirymptome.de)
- whonamedit.com: Emile Charles Achard (English)
- whonamedit.com: Joseph Thiers (English)
Individual evidence
- ^ EC Achard and J. Thiers: Le virilisme et son association à l'insuffisance glycolytique (diabète des femmes à barbe). In: Bull. Acad. Méd. 86, 1921, pp. 51-66.
