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.
Hematological
- Hemophilia A
Hemophilia B Hemorrhagic Telangiectasia, Hereditary Henoch-Schnlein Purpura Hepatic Encephalopathy Hepatic Fibrosis, Congenital Hepatitis B Hepatitis C Hepatorenal Syndrome Hereditary Exostoses, Multiple Hereditary Hyperphosphatasia Hermansky Pudlak [arrayit.com]
Entire Body System
- Virilization
21-hydroxylase deficiency, virilizing, non salt wasting 21-hydroxylase deficiency, virilizing, salt losing 3 beta-hydroxysteroid dehydrogenase deficiency Adrenal hyperplasia, congenital Adrenal hyperplasia, congenital, simple virilizing Adrenal hyperplasia [icd9data.com]
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]
- Weight Loss
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]
I know that 5:2 Diet gives you not only desired results (weight loss), but, also, it has a positive influence on your health. [trydietbooster.co.uk]
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]
The common symptoms of Achard-Thiers syndrome are: Onset of diabetes Frequent urination Excessive thirst and hunger Weight loss The signs and symptoms caused by overproduction of androgens may include: Hirsutism : Development of excess body hair on the [hellodoktor.com]
- Infertility
[…] 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 [symptoma.com]
The use of laparoscopy in amenorrhea and in the infertile female is both of gynecologic and endocrinologic interest, and its diagnostic value has been recently reviewed by Cohen (1970). [eje.bioscientifica.com]
[…] cells Pericanalicular sclerosis Liver cirrhosis Colloid changes of thyroid Hirsutisms Hypertension Amenorrhoea Hirsutism: Development of excess body hair on the face, chest, and back Receding hairline Deepening of the voice Enlargement of the clitoris Infertility [healthinfi.com]
Excessive thirst and hunger Weight loss The signs and symptoms caused by overproduction of androgens may include: Hirsutism : Development of excess body hair on the face, chest, and back Receding hairline Deepening of the voice Enlargement of the clitoris Infertility [hellodoktor.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]
Gastrointestinal
- 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]
Cardiovascular
- Hypertension
Clinical presentation based on findings of hirsutism, virilization, hypertension, and abnormally high serum androgen levels in postmenopausal females. [accessanesthesiology.mhmedical.com]
There were twenty subjects & most of them were selected because they have the following existing conditions: hirsutism, obesity, hypertension, angiopathy & overt diabetes. [signssymptoms.org]
Virilizing disorder characterized by voice changes, hypertrichosis of face, acne, enlarged clitoris, obesity, hypertension, small breasts, and abdominal striae. [insidesurgery.com]
Patients may also have amenorrhoea, hypertension and osteoporosis. [comfortfinders.com]
Skin
- Hirsutism
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. [dx.doi.org]
There were twenty subjects and most of them were selected because they have the following existing conditions: hirsutism, obesity, hypertension, angiopathy, and overt diabetes. [dianamossop.com]
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]
Psychiatrical
- 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]
Urogenital
- Amenorrhea
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]
The use of laparoscopy in amenorrhea and in the infertile female is both of gynecologic and endocrinologic interest, and its diagnostic value has been recently reviewed by Cohen (1970). [eje.bioscientifica.com]
Complications Some of the complications of ATS can include: The absence of menstrual periods – Amenorrhea High blood pressure Weak or brittle bones that can fracture easily with minimal trauma – Osteoporosis Abnormal ovaries, atrophy or sclerosis of the [modasta.com]
Complications due to Achard-Thiers Syndrome may include: Amenorrhea: Absence of menstrual periods Hypertension Osteoporosis: Brittle or weak bones that can fracture easily after mild trauma Atrophy or sclerosis of the ovaries; abnormal ovaries Pancreatic [healthinfi.com]
Amenorrhea, hypertension, and osteoporosis are often present. Pathological findings include liver cirrhosis, atrophy or sclerosis of the ovaries, and an increase in the size of the islands of Langerhans. [whonamedit.com]
- Oligomenorrhea
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]
[…] sclerosis Liver cirrhosis Colloid changes of thyroid Hirsutisms Hypertension Amenorrhoea Hirsutism: Development of excess body hair on the face, chest, and back Receding hairline Deepening of the voice Enlargement of the clitoris Infertility Obesity Oligomenorrhea [healthinfi.com]
[…] symptoms and signs related to excess androgens in the body include: Hirsutism: Excess hair on face, body, chest, and back Male pattern hair growth in excess A deep voice like a male in women Obesity Infrequent or very light bleeding during periods – Oligomenorrhea [modasta.com]
[…] hunger Weight loss The signs and symptoms caused by overproduction of androgens may include: Hirsutism : Development of excess body hair on the face, chest, and back Receding hairline Deepening of the voice Enlargement of the clitoris Infertility Obesity Oligomenorrhea [hellodoktor.com]
- Polyuria
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]
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].
Urine
- Glycosuria
Dysregulation Rapp Hodgkin Syndrome Raynaud's Disease and Phenomenon Recurrent Respiratory Papillomatosis Reflex Sympathetic Dystrophy Syndrome Refractory Sprue Refsum Disease Relapsing Polychondritis Renal Agenesis, Bilateral Renal Cell Carcinoma Renal Glycosuria [arrayit.com]
Hereditary fructose intolerance Essential benign fructosuria Fructosemia 271.3 Intestinal disaccharidase deficiencies and disaccharide malabsorption Intolerance or malabsorption (congenital) (of): glucose-galactose lactose sucrose-isomaltose 271.4 Renal glycosuria [theodora.com]
Serum
- Hyperinsulinemia
Achard Thiers syndrome is an extremely rare endocrine disorder characterized by hyperandrogenism and hyperinsulinemia. [symptoma.com]
Women who have Achard-Thiers Syndrome will have high levels of insulin in their body, this is called hyperinsulinemia. [patientworthy.com]
The constellation of clinical androgen excess and failure of the blood sugar control system to work properly (hyperinsulinemia) is now commonly identified earlier in a woman’s life, typically during adolescence and young adulthood, as polycystic ovary [rarediseases.org]
Type 2 diabetes with insulin resistance, the body becomes resistant to natural or medicated insulin, pushing your body to produce more insulin which can also lead to hyperinsulinemia ( or excess of insulin in the body). [lybrate.com]
- Hyperglycemia
Hypoglycemia: NOS reactive spontaneous Excludes: hypoglycemia: with coma (251.0) in diabetes mellitus (250.8) leucine-induced (270.3) 251.3 Postsurgical hypoinsulinemia Hypoinsulinemia following complete or partial pancreatectomy Postpancreatectomy hyperglycemia [theodora.com]
The most common manifestation observed in present study was cutaneous infections (39.66%), similar to Mahajan et al. [9] and Nigam et al. [10] It is well-known that diabetic patients are susceptible to infections, probably due to hyperglycemia and defects [ijem.in]
Association of GYSI and beta(3)-AR gene with postprandial hyperglycemia and serum uric acid in type 2 diabetes mellitus.// Chin. Med. J. (Engl).-2002.-Sep;l 15(9).-p. 1308-11 162. Waterworth D.M., Bennett S.T., Gharani N., et al. [dissercat.com]
- Insulin Increased
In the case of type 2 diabetes, your body becomes resistant to insulin increasing the level of the blood sugar level, making it difficult for the whole body to process. It generally happens due to lifestyle choices or mutated genetics. [lybrate.com]
Treatment
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]
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]
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]
Pathophysiology
Springer Science & Business Media, 06.12.2012 - 1853 Seiten Dermatology covers all the classical and related fields of dermatology, providing a wealth of information on clinical features, pathophysiology, and differential diagnosis. [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]
Prevention Mental and nutritional counseling is help to avoid or control mental stress and physiologic stress and also blood pressure. Some times it may be genetic or inherited. [checkorphan.org]
References
- 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.
- 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.
- Dunaif A. Insulin resistance and the polycystic ovary syndrome: mechanism and implications for pathogenesis. Endocr Rev. 1997;18:774-800.
- Adashi EY. Hyperinsulinemic Androgenism: A Pathophysiologic Paradox. In: R. J. Chang (ed.), Polycystic Ovary Syndrome. Serono Symposia USA book series; 1996:245-253.
- 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.