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Achard Thiers Syndrome

Diabetic Bearded Women Syndrome

Achard Thiers syndrome is an extremely rare endocrine disorder characterized by hyperandrogenism and hyperinsulinemia. It is primarily seen in postmenopausal women and was initially described as "diabetes mellitus in bearded women", reflecting the excess androgen manifesting as hirsutism. Additional symptoms include polyuria, polydipsia, weight loss, and menstrual irregularities - oligomenorrhea and amenorrhea. The diagnosis rests on a thorough clinical and biochemical assessment. The oral glucose tolerance test (OGTT) and a hormonal workup are crucial for discriminating this syndrome from classical diabetes mellitus or other hormonal disorders.


Presentation

Described almost 100 years ago, Achard Thiers syndrome is now rarely mentioned in the literature, primarily because of its infrequent occurrence, underdiagnosis, and poor understanding of the pathogenesis [1] [2] [3] [4]. Some of the first authors who described it, termed the disorder "diabetes mellitus of bearded women", as women who suffered Achard Thiers syndrome had simultaneous problems with glucose metabolism and adrenocortical hormones (the name hyperinsulinemic hyperandrogenism is also mentioned in some studies) [1] [2] [3]. The clinical presentation of Achard Thiers syndrome is comprised of the main symptoms of diabetes on one hand (polyuria, polydipsia, etc.), and signs of hyperandrogenism on the other (hirsutism, especially on the head and neck; menstrual changes such as oligomenorrhea and amenorrhea, clitoral enlargement, infertility, and obesity) [5]. Postmenopausal women are identified as the main patient population [3] [5]. Some studies have emphasized the primary role of adrenocortical disease in the etiology of insulin resistance seen in patients with Achard Thiers syndrome, and have remarked that diabetes mellitus is not related to the changes that normally develop in the pancreas [4] [5]. In addition, psychiatric symptoms and disorders were noted in the first patients described with this condition [5]. Acanthosis nigricans is a common symptom of Achard Thiers syndrome as well. Another condition characterized by hyperandrogenism and hyperinsulinemia is the polycystic ovary syndrome or PCOS, which is usually identified in young women.

Virilization
  • - Feminisation-adrenogenital - Feminization-adrenogenital - Virilization-adrenogenital - Adrenogenital disorders (& [feminisation] or [virilisation] or [Achard-Thiers syndrome]) Hide descriptions.[snomedbrowser.com]
  • Achard-Thiers syndrome One form of a virilizing disorder of adrenocortical origin in women, characterised by masculinization and menstrual disorders in association with manifestations of diabetes mellitus, such as glucosuria. ... (05 Mar 2000) ...[encyclo.co.uk]
  • Achard-Thiers syndrome - one form of a virilizing disorder of adrenocortical origin in women. Thiers, Joseph, French physician, 1885–. Achard-Thiers syndrome - see under Achard[medical-dictionary.thefreedictionary.com]
  • Virilizing disorder characterized by voice changes, hypertrichosis of face, acne, enlarged clitoris, obesity, hypertension, small breasts, and abdominal striae.[insidesurgery.com]
Weight Gain
  • Pregnancy causes weight gain and increases levels of estogen and placental hormones, which antagonize insulin. some medications such as thiazide diuretics, adrenal corticosteroids, and hormonal contraceptives are also resulted Achard-Thiers syndrome.[checkorphan.org]
Camping
  • Achard Legumes 100 0 0.02 Achard Mangue 100 0.12 0.01 Les Achards 100 0 0.0 Thiers Coutellerie 100 1.96 0.27 Barbara Thiering 100 0.69 0.09 Autocaz Thiers 100 2.73 0.08 Louis Thiers 100 0.02 0.09 Thier Münster 100 0.26 0.14 Campanile Thiers 100 6.42 0.28 Camping[minikeyword.com]
Polydipsia
  • Additional symptoms include polyuria, polydipsia, weight loss, and menstrual irregularities - oligomenorrhea and amenorrhea. The diagnosis rests on a thorough clinical and biochemical assessment.[symptoma.com]
Hirsutism
  • It is primarily seen in postmenopausal women and was initially described as "diabetes mellitus in bearded women", reflecting the excess androgen manifesting as hirsutism.[symptoma.com]
  • Williams : Hirsutism in females. A clinical study of its aetiology, cause and treatment. Ann. Int. Med. 22, 773–806 (1945). Google Scholar 8. Callow, N.H., R.K. Callow and C.W.[doi.org]
  • Pierre University of Brussels Belgium Summary Twenty patients, selected on the basis of simultaneous existence of overt diabetes and marked hirsutism, were submitted to clinical and biological investigations.[deepdyve.com]
Receding Hairline
  • hairline Deepening of the voice Enlargement of the clitoris Infertility Obesity Oligomenorrhea: Infrequent or very light menstrual periods Amenorrhea: Absence of menstrual periods Facial hypertrichosis (excess hair on the face in males) Adrenal cortical[healthinfi.com]
  • Other signs of the syndrome are directly due to the overproduction of androgens, and may include an increase in body hair, particularly on the face, chest, back, and other areas, receding hairline, deepening of the voice, enlargement of the clitoris;[rarediseases.org]
Psychiatric Symptoms
  • In addition, psychiatric symptoms and disorders were noted in the first patients described with this condition. Acanthosis nigricans is a common symptom of Achard Thiers syndrome as well.[symptoma.com]

Workup

A detailed patient history and a thorough physical examination are the two crucial steps needed for the diagnosis of Achard Thiers syndrome. Physicians should inquire about the onset of abnormal hair growth and evaluate the onset, progression, and severity of additional symptoms. The physical examination should confirm the previously mentioned findings and provide solid evidence for a detailed laboratory investigation. The OGTT test is sufficient to detect hyperinsulinemia, while a complete androgen hormone panel is recommended for assessing the adrenal gland function. Because obesity and hypertension are frequently present, a comprehensive cardiologic examination through blood pressure measurements and electrocardiography (ECG) is often made. Imaging studies, such as ultrasonography or computed tomography (CT) may be of great benefit in determining the underlying cause of Achard Thiers syndrome, as intrinsic adrenocortical pathologies (e.g. carcinoma) have been described in its pathogenesis [5].

Glucose Decreased
  • An effective treatment should normalize the blood glucose & decrease the complications through the use of insulin replacement, exercise & proper diet.[signssymptoms.org]

Treatment

  • An international group of leading experts addresses the varied etiologies of PCOS, comprehensively covering the contemporary treatment approaches and long-term implications of PCOS, a common yet poorly understood disorder.[books.google.com]
  • Treatment : How to Treat "achard-thiers syndrome"? The most effective treatment for Achard Thiers syndrome is to treat the underlying cause which is diabetes mellitus.[signssymptoms.org]
  • The information presented is not intended to replace medical advice or treatment from your own doctor or healthcare provider.[wellnessadvocate.com]
  • The diagnosis of Achard-Thiers Syndrome is based on the symptoms manifested by the disorder The treatment of Achard-Thiers Syndrome includes managing diabetes and the use of hormonal therapies.[healthinfi.com]

Prognosis

  • Keeping the blood sugars, and blood pressure in control and stopping smoking can result in lower rates of complications and a better prognosis. Consult a top Diabetologist Copyright 2018 Modasta. All rights reserved CheckOrphan.[modasta.com]
  • Prognosis - Achard-Thiers syndrome Not supplied. Treatment - Achard-Thiers syndrome Normalises blood glucose levels by using insulin,anti diabetic drugs or exercise or through diet.[checkorphan.org]

Etiology

  • An international group of leading experts addresses the varied etiologies of PCOS, comprehensively covering the contemporary treatment approaches and long-term implications of PCOS, a common yet poorly understood disorder.[books.google.com]
  • Some studies have emphasized the primary role of adrenocortical disease in the etiology of insulin resistance seen in patients with Achard Thiers syndrome, and have remarked that diabetes mellitus is not related to the changes that normally develop in[symptoma.com]
  • (Etiology) Achard-Thiers Syndrome appears to be transmitted within families. But the exact mechanism of genetic transmission is not yet understood.[dovemed.com]

Epidemiology

  • Epidemiological profile revealed almost equal incidence in males and females (51% and 49%, respectively) with 51.25 14.42 (mean SD) years of age.[ijem.in]
Sex distribution
Age distribution

Pathophysiology

  • Presenting an overview of the current understanding of the pathophysiology of PCOS and a paradigm for the clinical evaluation and management of the disorder, Polycystic Ovary Syndrome is written in an easy to digest, concise format that, with bulleted[books.google.com]
  • The pathophysiologic roles of interleukin-6 in human disease (An edited summary of a Clinical Staff Conference held on 13 March 1996 at the National Institutes of Health, Bethesda, MD). // Ann. Intern. Med.-1998.-128.-p.127—37 24.[dissercat.com]

Prevention

  • Prevention Some of the measures that can be helpful in preventing ATS include: Maintaining ideal body weight Dietary modification and weight reduction to control blood pressure and diabetes Regular exercises to prevent obesity Avoid smoking to reduce[modasta.com]
  • Currently, there are no definitive methods available to prevent Achard-Thiers Syndrome.[healthinfi.com]
  • These are important contributing factors for Achard-Thiers syndrome Doing regular exercise will help to prevent obesity If you have any questions, please consult with your doctor to better understand the best solution for you.[hellodoktor.com]

References

Article

  1. Vahora R, Thakkar S, Marfatia Y. Skin, a mirror reflecting diabetes mellitus: A longitudinal study in a tertiary care hospital in Gujarat. Indian J Endocrinol Metab. 2013;17(4):659-664.
  2. Malaisse W, Lauvaux JP, Franckson JR, Bastenie PA. Diabetes in bearded women (Achard-Thiers-Syndrome) : A clinical and metabolic study of 20 cases. Diabetologia. 1966;1(3-4):155-161.
  3. Dunaif A. Insulin resistance and the polycystic ovary syndrome: mechanism and implications for pathogenesis. Endocr Rev. 1997;18:774-800.
  4. Adashi EY. Hyperinsulinemic Androgenism: A Pathophysiologic Paradox. In: R. J. Chang (ed.), Polycystic Ovary Syndrome. Serono Symposia USA book series; 1996:245-253.
  5. R. Azziz. A Brief History of Androgen Excess. In: R. Azziz et al, eds. Contemporary Endocrinology: Androgen Excess Disorders in Women: Polycystic Ovary Syndrome and Other Disorders. 2nd Ed. Totowa, NJ: Humana Press Inc.; 2004:3-16.

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Last updated: 2019-06-28 10:42