What is the Achard-Thiers syndrome?The main cause of Achard-Thiers syndrome is an adrenocortical tumor (see picture) or a basal pituitary adenoma.
Achard-Thiers syndrome is a disease characterized by the characteristics of the trunk, hypertrichosis and diabetes mellitus. The name of the syndrome derives from Emile Achard and Joseph Thiers.
The two French physicians described the disease for the first time in 1921 and awarded him because of the two most striking features the term "diabetes bearded women", which is considered obsolete today.
Achard-Thiers syndrome is not an isolated clinical phenomenon but is based on a different disease. Typically, this is a tumor that is either a basophilic pituitary adenoma or an adrenocortical tumor. Basophilic pituitary adenoma affects the pituitary gland (pituitary gland) is one of the benign tumors, but may affect human functions.
Some pituitary adenomas produce hormones; Therefore, depending on the type of hormones they secrete, the medicine also divides these tumors into different types. The other type of tumor that may be the cause of Achard-Thiers syndrome is the adrenal cortex.
Like the pituitary gland, the adrenal cortex plays an important role in the hormonal regulation of the organism. Both can therefore cause an imbalance in the composition and concentration of hormones, which among other things has the beard growth, which is so conspicuous in this syndrome.
Symptoms, complaints & signs
A striking symptom of Achard-Thiers syndrome is the beard growth (hirsutism) of the affected women, which is due to an increased concentration of male sex hormones in the female body. These are also known as androgens and include, among others, testosterone, androsterone, corticosterone and others.
However, beard growth in women or hirsutism can also be attributed to other causes and, like the other symptoms, is not a unique feature of Achard-Thiers syndrome. At the same time, it is possible for the hair to fall out on other parts of the body, for example on the head. In addition, sufferers suffer from diabetes mellitus; The disease is also popularly known as "diabetes" and is a metabolic disorder that leads to a faulty regulation of the energy balance in the human body.
Achard-Thiers syndrome is typically associated with adiposity (obesity) and arterial hypotension (elevated blood pressure). In addition, in women, the disease can lead to the absence of menstruation; The medicine refers to this condition as amenorrhea. In some cases, patients with Achard-Thiers syndrome may experience an enlarged clitoris, either present from birth or later in life.
Diagnosis & History
Doctors typically diagnose Achard-Thiers syndrome based on clinical presentation, as it is characterized by a combination of various symptoms. The beard growth (Hirsutismus) is externally well visible and does not require any elaborate diagnostics. A special blood test can usually make diabetes mellitus visible.
The cause of Achard-Thiers syndrome is a pituitary adenoma, the most common side effects are the occurrence of diabetes mellitus, obesity, absence of menstrual bleeding, hair loss and hirsutism. The pituitary adenoma can be treated with medication or surgery. In the case of surgery, there are general complications, such as thrombosis, pulmonary embolism or wound healing disorders.
If an Achard-Thiers syndrome remains untreated, the numerous side effects often result in considerable complications. Those affected by diabetes mellitus, for example, suffer from damage to the eyes or kidneys; in the worst case, a diabetic foot or a diabetic coma may be at risk. Obesity (obesity) leads untreated to numerous health restrictions: cardiovascular diseases, damage to bones and joints, forming a diabetes type II.
The overproduction of male hormones often leads to fertility problems (fertility), polycystic ovaries in combination with the absence of menstrual periods prevent the onset of pregnancy. For women with hirsutism (increased hair growth) or hair loss especially the psychological consequences of Achard-Thiers syndrome are significant, since the appearance of women with the usual ideal of beauty does not harmonize.
In general, it can be observed that the psychological effects of untreated Achard-Thiers syndrome, in particular, have considerable negative effects. A significant proportion of affected women suffer from depression to a complete retirement from social life.
When should you go to the doctor?
This special type 2 diabetes mellitus is always in the hands of a doctor. Since the disease is relatively rare in this form, it should be a specialist. For example, an endocrinologist would be suitable. When a post-menopausal woman develops Achard-Thiers syndrome, usually a benign tumor is behind it.
With an endocrine complex disease like this is not to joke. Therefore, it is necessary to install a close monitoring of diabetes mellitus plus obesity and hair loss. If hypertension and overproduction of male sex hormones are also present, the suspicion of a tumor on the pituitary gland or the adrenal cortex is close. The problem is that the individual symptoms must be linked, rather than considered and treated individually.
Usually, however, it is seldom reported to the same doctor that in addition to the already existing diagnosis of diabetes mellitus, one also suddenly suffers from hair loss and increased beard growth. The obesity, however, is obvious to the doctor. However, whether the physician adds up one and one when the patient develops hypertension in addition to the other symptoms is questionable. An overproduction of progestagens is detected in a hormone test. This is caused by the gynecologist - but only if this sees a reason for a hormone test.
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Treatment & Therapy
The treatment of Achard-Thiers syndrome differs depending on the individual conditions of the disease. The syndrome is usually the result of another disease, the underlying cause; Accordingly, the treatment of this underlying disease also plays a crucial role in the therapy of Achard-Thiers syndrome.
Diabetes mellitus is only one aspect of many, and patients can support the drug therapy of diabetes through changes in their personal lifestyle. Exercise and a balanced, healthy diet are important decision-makers from this point of view.
One possible cause of Achard-Thiers syndrome is a basophilic pituitary adenoma. In such a case, an operative removal of the tumor is often in question. Depending on the type of adenoma, different drug treatment options may be considered, which in turn depend on the individual factors of the individual case.
Additional symptoms and consequences of basophilic pituitary adenoma also require appropriate treatment. For example, it is possible that as a result of the tumor, an overactive thyroid develops when the tumor forms corresponding hormones. Under circumstances, doctors treat them with other drugs that aim to normalize the level of thyroid hormone levels again.
If the Achard-Thiers syndrome underlying a tumor in the adrenal cortex, comes in many cases, an operative treatment into consideration. During this procedure, if possible, clinicians will completely remove the adrenal cortex tissue, leaving as much healthy tissue untouched as it appears safe.
Since the adrenal cortex occupies an important position in the hormone balance of the human organism and among other things, for the cerebral metabolism of importance, then patients may take appropriate medication. These are intended to replace the failing or impaired hormonal performance of the adrenal cortex tissue.
Outlook & Forecast
The Achard-Thiers syndrome leads to very unpleasant complications. Especially women are affected by the syndrome of a strong beard growth. This often leads to inferiority complexes and reduced self-esteem. Some sufferers are also depressed and suffer from other mental health problems. However, hair can also be lost on other regions of the body or it can lead to diabetes.
Due to the disturbance of the metabolism also obesity occurs, which leads to further complaints and to a restriction of movement. The heart is also adversely affected by obesity. The blood pressure is increased and the risk of a heart attack increases rapidly.
In most cases, an early diagnosis is possible, so that the treatment of Achard-Thiers syndrome can be started early. Diabetes can damage kidneys, eyes and feet. In many cases, the treatment itself does not lead to further complications and can limit the symptoms relatively well, resulting in a positive course of the disease. However, it is not always possible to counteract the male, so it comes with many sufferers to severe depression.
Since the Achard-Thiers syndrome is not an independent disease, but occurs in the context or as a result of an underlying disease, a specific prevention for this syndrome seems difficult. In the foreground is the treatment of the underlying disease, which is usually a tumor.
Measures that patients can take on their own concern, above all, the regular and correct intake of medicines as well as the participation in other forms of treatment and general positive health measures, such as a balanced diet.
Since Achard-Thiers syndrome is a hereditary disease, it can not be treated causally but only symptomatically. For this reason, the possibilities of aftercare are very limited and hardly possible. Even complete cure of Achard-Thiers syndrome is usually not achieved.
Since the Achard-Thiers syndrome is hereditary, in the case of an existing desire to have a child, genetic counseling can be useful in order to prevent the transmission of the syndrome to the children. The disease is usually treated with medication.
The patient should pay attention to the regular use of these drugs, while also interactions with other medications taken should be considered. Furthermore, the affected person's thyroid gland must also be monitored regularly to prevent malfunctioning and further complications. Furthermore, the Achard-Thiers syndrome can also significantly increase the likelihood of a heart attack.
For this reason, regular examinations of the heart make sense, and even a healthy lifestyle with a healthy diet can have a very positive effect on the course of the disease. Likewise, contact with other Afflicted Achard-Thiers syndrome can provide valuable information that can contribute to an improvement of the condition.
You can do that yourself
The symptoms of Achard Thiers syndrome vary greatly from patient to patient. They also depend on the underlying disease. Having them treated is of paramount importance. While the underlying disease may differ, a negative impact on appearance is usually a commonality. The ladybear is part of the disease.
Nevertheless, to maintain a positive attitude is important for the ill women. A life-affirming attitude counteracts the threat of social withdrawal and depression. An exchange with other patients can also be supportive of therapy. Openness in dealing with the disease, active participation in social and family life are cornerstones in self-help. Equally important is the lifelong and disciplined intake of prescribed medication. In addition, psychotherapeutic conversations can have a positive effect.
As a rule, the Achard-Thiers patients suffer from diabetes. The effects of diabetes are well manageable when the lifestyle is adjusted. A change in diet to a full-fledged diet makes sense, as well as the integration of sport into everyday life. Since the patients often also suffer from obesity and hypertension, especially regular exercise is beneficial. The visual impairment of the disease can be significantly improved by skillful choice of clothes, hair replacement and specially trained makeup techniques.