Definition
Definition
Acromegaly is a rare disease that mainly affects individuals aged 30 to 40, characterized by numerous symptoms, particularly an increase in volume (hypertrophy) of different regions of the body, the origin of which is not congenital, mainly affecting the extremities and the head.
Generalities
We also note a hypertrophy bone tissue, many organs, ears, nose, language.
In children, it causes a gigantism.
Symptoms
Symptoms
- An increase in the volume of the feet, skull, nose, ears, tongue, heart, thyroid, etc. accompanied by:
- On the prognathism (protrusion forward of the lower jaw).
- Spinal deformities (Scoliosis, kyphosis).
- From a thickening of the features.
- From a detachment of the ears.
- From a gap between the teeth.
- In hypertension (increased blood pressure).
- In hyperglycemia (increased blood sugar level).
- Psychological problems.
- In a hollow voice.
- In asthenia (intense fatigue) accompanied by a feeling of great weariness.
- From drowsiness.
- Night sweats.
- Headaches (headaches).
- Tingling in the hands.
- Pain in the bones of the joints (quite frequently).
Medical exam
Physical examination
It shows the presence of a kyphosis of the segment of the spine, concerning the cervical vertebrae, and the dorsal vertebrae, associated with a protrusion of the sternum (we are talking about a double punch).
A kyphosis is a deformity in the shape of a arch of the spine: angulation with posterior convexity (hump), that is to say whose curvature looks backwards.
Kyphosis usually affects the dorsal column (part of the spine corresponding to the back, between the shoulder blades).
Normally, there is a physiological (normal) convexity at this level which tends to become excessive in kyphosis.
This results in a deformation of the whole body, giving the patient the silhouette of an individual with the neck projected forward.
Labo
The dosage of the different hormones secreted by the pituitary gland will allow the diagnosis to be made, it concerns:
- L' Growth Hormone (GH) deficiency.
- La Prolactin : protein hormone, produced by the pituitary gland, and more particularly its anterior lobe (part of the pituitary gland located at the front).
- La TSH.
- La gonadotropin (hormone whose action affects the genitals).
- ACTH (hormone having an action on the secretion of cortisol : natural cortisone).
Additional examination
Additional examinations and more particularly the computed tomography (scanner) as well as the'MRI., easily show adenoma of the pituitary gland.
Ophthalmological examinations allow the study of the visual field and checks that there is no compression of the optical channels (optic nerve).
Cause
Cause
Acromegaly is due to excessive secretion of growth hormone, caused by a benign tumor (eosinophilic adenoma of the anterior lobe) of the pituitary gland.
The pituitary gland is a small gland considered to be the conductor of the other hormonal glands, because it has a regulating action on it. The pituitary gland itself receives orders from the hypothalamus, an area located in the center of the brain, and possessing both neurological and hormonal properties. adenomas Pituitary gland tumors account for about 10 to 15% of tumors located inside the skull. They are likely to cause endocrine imbalance (hormonal) of the front part of the pituitary gland (anterior pituitary). They can also affect neighboring structures. Pituitary adenomas are, after thyroid tumors, the most common endocrine (hormonal) tumors. They are likely to cause compression of neighboring organs, and a endocrine syndrome specific (disorder of the body in direct relation to the affected hormone). There are various types of pituitary adenomas, the classification of which depends on histological analysis (analysis of the tissues constituting the tumor). The symptoms of pituitary adenoma are linked either to a modification of the secretion of hormones, which is either increased or decreased (hypopituitarism, linked to a so-called adenoma chromophobe).
Depending on the type of pituitary tumor, the patient may experience:
- The headache (headaches) located behind the eyeball, at the temples.
- The eye disorders (double vision among others).
- A galactorrhea amenorrhea (absence of periods associated with abnormal secretion of milky fluid from the nipples).
- An enlargement of the organ which is due to an abnormally high secretion of somatotropin.
- Growth retardation due to insufficiency anterior pituitary (front part of the pituitary gland).
- A deficiency in sex hormones leading to genital disorders.
- A bitemporal hemianopia (the subject no longer sees to the sides).
Treatment
Treatment
Medical treatment uses bromocriptine, a dopamine-like drug.
Side effects of bromocriptine (which is given in small amounts at the beginning of treatment) include:
- Nausea.
- Stomach irritation.
- A orthostatic hypotension (drop in blood pressure when the individual suddenly moves from a lying position to a standing position).
- Headaches.
- A fatigue.
- Abdominal cramps.
- Nasal congestion.
- Constipation.
- Octreotide is the complementary treatment used in acromegaly. It is sometimes also used in preparation for surgery. Surgical treatment is used when the visual field impairment does not regress quickly (e.g., after 1 to 3 months of bromocriptine). Surgery most often corrects the excess hormonal secretion. radiotherapy (use of rays for therapeutic purposes) is effective in preventing a tumor growth, but its results are unsatisfactory in the acute treatment of pituitary hyperfunction. More recently, analogues of the somatostatin, which is a hormone secreted mainly in the digestive tract at the level of endocrine (hormonal) cells belonging to the duodenum and pancreas. It is also present in the central nervous system and more particularly at the level of the hypothalamus (see above). The role of somatostatin is to inhibit digestive secretion, and other hormones both endocrine (secreted into the blood) and exocrine (secreted directly into the digestive tract). Its role is also to inhibit the secretion of growth hormone by the pituitary gland. The trade name for somatostatin analogues is Sandostatin, which is used as an adjunct to surgical treatment of pancreatic tumors, pituitary adenomas and certain diarrheas. In some cases, it is necessary to remove the tumor, supplemented by radiotherapy. The treatment allows a reduction of excess secretion of pituitary hormone in approximately half of people with a large adenoma.
Evolution
Evolution
The complications of acromegaly are mainly rheumatic.
This is how we observe in certain patients:
- The kyphoscoliosis (bumps) described previously.
- The pain articular diffuse.
- Damage to the heart and blood vessels that can cause heart pump failure, sometimes severe.
- De visual disturbances with a risk of compression of the optic nerve when the pituitary adenoma grows too large.