Achard Thiers Syndrome

Achard Thiers Syndrome

Achard-Thiers syndrome is a rare disorder that occurs primarily in postmenopausal women and is characterized by type 2 (insulin-resistant) diabetes mellitus and signs of androgen excess. The exact cause of this syndrome is unknown.

Overview

Achard-Thiers syndrome is a hormonal disorder that occurs in diabetic postmenopausal women. It is characterized by diabetes mellitus and hirsuitism. It combines the features of Adrenogenital syndrome and Cushing syndrome. It is also known as diabetic-bearded woman syndrome.

Symptoms

• 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

Causes

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

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

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.

Related Disorders

Symptoms of the following disorders can be similar to those of Achard-Thiers. Comparisons may be useful for a differential diagnosis:

Acquired adrenogenital syndrome is a rare endocrine disorder that occurs because of a tumor in the adrenal glands which causes the overproduction of androgens. In an adult female the symptoms may include a male pattern of hair growth, loss of hair on the head, acne, deepening voice, and abnormally large muscles.

Empty sella syndrome is a rare brain disorder that may be inherited or acquired. The acquired form of the disease may occur as a result of a tumor in the pituitary gland or radiation to that area. Symptoms may include headaches, impaired vision, and/or obesity. Some people with empty sella syndrome cannot tolerate cold temperatures and may also have abnormally high blood pressure (hypertension). In women this disorder is associated with an increase in body hair and a male pattern of hair growth. (For more information on this disorder, choose “Empty Sella” as your search term in the Rare Disease Database.)

Diabetes is a complex endocrine disease that occurs when the pancreas does not produce enough insulin or the body is not able to use insulin properly. There are two main groups of diabetes: Type I (insulin-dependent) and type II (noninsulin-dependent). The symptoms of diabetes usually include frequent urination, extreme thirst, constant hunger, and unexplained weight loss. Long-term complications of diabetes may affect many organs of the body including the nervous system, heart, kidneys, and eyes. (For more information on this disorder, choose “Diabetes” as your search term in the Rare Disease Database.)

Polycystic ovary syndrome (PCOS) affects women and is a complex of symptoms that are not necessarily all present in all cases. Some, but not all, affected women have multiple cysts on the ovaries. Other characteristics include absent or irregular menstruation, failure of the ovary to release eggs (anovulation), elevated levels of the male hormones known as androgens (hyperandrogenism), excessive amounts of body hair (hirsutism), a high rate of miscarriage, and infertility. Three criteria often used for a diagnosis are menstrual irregularity, hyperandrogenism, and exclusion of other disease. There is some evidence that PCOS is an inherited condition.

Diagnosis

The diagnosis of Achard-Thiers syndrome should be suspected based on the clinical findings. Because affected women are hyperinsulinemic, a two-hour oral glucose tolerance test shows abnormally elevated levels of glucose in the blood.

Diabetes may be managed by diet and/or insulin or other medications, as required. Cosmetic measures (for example, waxing and electrolysis) can be used to facilitate hair removal. For younger women with PCOS, treatment with an oral contraceptive is the most common therapy, whereas for postmenopausal women with Achard-Thiers syndrome, hormone replacement therapy is usually recommended. Antiandrogens have also been used.