About Achard Thiers Syndrome


Achard Thiers Syndrome

Achard–Thiers syndrome combines the features of adrenogenital syndrome and Cushing syndrome. It is also known as diabetic-bearded woman syndrome (diabète des femmes à barbe) and occurs mainly in post-menopausal women. Achard-Thiers Syndrome is a rare, uncommon hormonal disorder that primarily affects postmenopausal women. It is also known as Diabetes in Bearded Women

The syndrome is characterized by the presence of the following features:

  • Type 2 diabetes (high sugar level)
  • Excess androgen
  • Hirsutism: Excessive growth of facial or body hair in women
  • Deep masculine voice
  • Acne
  • Enlarged clitoris
  • Obesity

Hyperplasia or adenoma of the adrenal gland. The adrenal glands are small, paired glands present atop the kidneys This syndrome appears to be transmitted within families, but the exact mechanism of genetic transmission is not yet discovered. 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. With appropriate and effective treatment the prognosis of the condition is very good

Diabetes in bearded women, Diabetic-bearded woman syndrome

  • Excess hair male pattern hair growth in females
  • Diabetes mellitus
  • Voice changes
  • Absent menstruation
  • Sparse menstruation
  • Excessive hair growth
  • Facial acne
  • Hypertrophic clitoris
  • Atrophic breast
  • Obesity
  • Abdominal striae
  • Increased blood pressure
  • Hyperplasia of adrenal glands
  • Adenoma of adrenal glands
  • Atrophic ovaries
  • Sclerotic ovaries
  • Pancreatic changes
  • Increased islet 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
  • Obesity
  • Oligomenorrhea: Infrequent or very light menstrual periods
  • Amenorrhea: Absence of menstrual periods
  • Facial hypertrichosis (excess hair on the face in males)
  • Adrenal cortical hyperplasia or adenoma

Causes – Achard-Thiers syndrome

Diabetes is the most important cause of this disease.The effects of diabetes mellitus result from insulin deficiency. Obesity contributes to the resistance to endogenous insulin. The Diabetics Mellitus result from various conditions,such a genetic defect of the beta cells or from use of certain drugs or chemicals,abnormal glucose during pregnancy. Physiologic or emotional stress can cause prolonged elevation of stress hormone levels(cortisol, epinephrine, glucagon,and growth hormone), this raises blood glucose levels,which in turn,places incresed demand on the pancreas. 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.

Prevention – Achard-Thiers syndrome

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. In this situation a family diet chart prepared which includes nutritional foods,Vitamin E and foods which normalize glucose levels. Maintain appropriate body weight is important. Certain drugs or chemicals may cause diabetics, stop that medicines.Control the glucose levels during pregnancy and avoid increased weight and placental hormones. Cholesterol and triglyceride levels may also be reduced.

Reduce smoking will help to avoid complications and prevent cardiovascular disease related with Achard-Thiers syndrome. Dietary education and weight reduction is important to control blood pressure and diabetes. The diet must be followed consistently and meals eaten at regular times. Exercise will help to control weight.

Treatment – Achard-Thiers syndrome

Normalises blood glucose levels by using insulin,anti diabetic drugs or exercise or through diet. Pancreas transplantation is expirimental and requires chronic immunosuppression. Treating diabetics is important. Sulfonylureas,Meglitinides,Biguanides, Alpha-glucosidase inhibitors, thiazolidinediones etc.are the drugs used to treat diabetes. These drugs also reduce insulin resistance by decreasing hepatic glucose production and increasing glucose uptake. Treatment of long-term diabetic complications may include transplantation or dialysis for renal failure, photocoagulation for retinopathy, and vascular surgery for large-vessel disease. Hyperbaric Oxygen therapy is needed for patients with wounds.

What are the Signs and Symptoms of Achard-Thiers Syndrome?

The common signs and symptoms associated with Achard-Thiers Syndrome include:

  • 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 face, chest, and back
  • Receding 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 hyperplasia or adenoma

What are the Risk Factors for Achard-Thiers Syndrome? (Predisposing Factors)

The risk factors associated with Achard-Thiers Syndrome include:

  • Postmenopausal women
  • Obese or overweight women

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider

How is Achard-Thiers Syndrome Diagnosed?

Achard-Thiers Syndrome is diagnosed using the following methods:

Complete physical exam with thorough medical history: The diagnosis is based on the symptoms manifested by the condition

The disorder can be confirmed by a 2-hour oral glucose tolerance test, which is done to check abnormally elevated glucose levels in blood

  • Blood levels of androgen, estrogen, hemoglobin A1c
  • Complete blood count
  • Thyroid function test
  • Liver function test
  • Ultrasound scan of abdomen
  • CT abdomen

What are the possible Complications of Achard-Thiers Syndrome?

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 defects
  • Abnormal liver function, liver cirrhosis
  • Signs and symptoms related to thyroid gland function

How is Achard-Thiers Syndrome Treated?

The various treatment methods for Achard-Thiers Syndrome include:

  • Diabetes may be managed through proper diet or intake of insulin or other related medications such as sulfonylureas, meglitinides, biguanides, alpha-glucosidase inhibitors, and thiazolidinediones
  • Cosmetic measures, such as waxing and electrolysis, may be helpful in removing excess hair
  • Hormone replacement therapy is often prescribed for post-menopausal women with Achard-Thiers Syndrome
  • Anti-androgens can also be used to treat symptoms of androgen excess
  • Hormonal therapy to rectify endocrine imbalances caused by systemic diseases may also be considered

How can Achard-Thiers Syndrome be Prevented?

Currently, there are no definitive methods available to prevent Achard-Thiers Syndrome. However, the following measures can be helpful:

  • Maintaining the correct body weight is important
  • Avoiding drugs that may cause diabetes
  • During pregnancy, care should be taken to control glucose levels and ensure proper weight management
  • Reducing cholesterol and triglyceride levels can also be beneficial
  • Reducing smoking will help to avoid complications and prevent heart diseases
  • Dietary education and weight reduction are very important factors in controlling blood pressure and diabetes. These are important contributing factors for Achard-Thiers Syndrome
  • Doing regular exercise will help to prevent obesity.

What is the Prognosis of Achard-Thiers Syndrome? (Outcomes/Resolutions)

With proper and effective treatment the prognosis of Achard-Thiers Syndrome is very good

  • Maintaining normal glucose levels will help in preventing complications such as retinopathy, nephropathy, and neuropathy
  • Controlling diabetes, blood pressure, and reducing or stopping smoking can result in lesser complications and a better prognosis

Additional and Relevant Useful Information for Achard-Thiers Syndrome:

  • Emile Charles Achard and Joseph Theirs were the first to describe the syndrome, and hence, the disorder is named after them
  • Disorders that are related to Achard-Thiers Syndrome include:
  • Acquired adrenogenital syndrome
  • Empty Sella Syndrome
  • Diabetes
  • Polycystic ovary syndrome

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