The clinical picture of acromegaly
Endocrinology / / May 14, 2016
Acromegaly is the most common disease of the anterior pituitary.Due to the slow course of the disease it is difficult to judge the true prevalence of acromegaly.The Cleveland Clinic revealed 1 case per 5850 primary patients.It is believed that acromegaly occurs with equal frequency in men and women.However, among the 145 patients passed through the Clinic-Union Institute of Experimental Endocrinology, 95 were women.In the group of patients, observed a group of doctors, women accounted for 61%.
Acromegaly occurs more often in middle age - after 30 years, to be observed in younger and older people.On the question of whether to develop acromegaly in children and adolescents up to puberty, the opinions of various authors differ.Eosinophil pituitary adenoma in children in general are very rare, and if they come to the age of the epiphyseal cartilage ossification, they do not cause acromegaly and gigantism.However, a description of a child 9 years 4 months, who had eosinophilic pituitary adenoma and, along w
acromegaly patients impose a variety of complaints.Often there are headaches ,: according to - in 87% of patients.Headaches can be mild, transient, but sometimes they are permanent and very strong.The most frequent localization of - the frontal and temporal regions.Tie persistent headaches in acromegaly with the pressure of the tumor on the diaphragm sella.We observed the patients complained of severe headache with no obvious signs of a tumor, and after removal of pituitary adenoma.
often appear relatively early paresthesia and burning pain in the hands.In some patients, the pain in his hands are the first symptom, forcing seek medical advice.
In connection with developing osteoarthritis are pain in the joints in the lower back.
Changing the appearance of patients due to the slow development of the disease is often described by patients and their surrounding is very late.Most patients pay attention to the growth of the limbs, in particular the need to increase the size notice shoes.
Often the first symptom of the disease, forcing the patient to see a doctor, is a violation of the menstrual cycle in women.
In the later stages of the disease, patients complain of muscle weakness, fatigue.
number of subjective disturbances may occur in connection with compression of the tumor surrounding tissue - blurred vision, photophobia, double vision, anosmia, tinnitus, vertigo.
Patients often made neurotic, anxious and somewhat depressed.
An objective study draws attention is very characteristic change in the face.The growth of the skull bones in acromegaly is uneven.Especially significantly increases the size of the lower jaw, which is massive, projecting forward (prognathism).
increases the angle between the horizontal and the ascending branches of the mandible.Changes bite - the teeth of the lower jaw protrude ahead of the upper;increased space between the teeth.Greatly increases the bone of the nose, cheek and brow ridges.The eyes are usually located deep in the hypertrophic eyebrows.Only a few patients have exophthalmos.Often they grow ears, tongue.Sprawl and soft tissue pastoznost in conjunction with a change in the facial skeleton create a distinctive image of a patient with acromegaly (see fig.).
This largely offset by individual traits, and acromegaly patients are strikingly similar to each other.
Dimensions cerebral part of the skull are also increasing, there is a proliferation of the occiput, resulting in increased head circumference.
skin in patients with acromegaly usually thickened, forming folds, especially on the face and sometimes - on the scalp.Often there is an increased pigmentation of the skin and moisture and greasiness of it due to hypertrophy of the sebaceous and sweat glands.The pores are often enlarged.However, in some patients the skin is dry or normal humidity.Hair thickened head.Often women appears hypertrichosis - vegetation on the face and body;observed as increased vegetation on the body of men.Hypertrichosis depends either from increased androgen production by the adrenal cortex, or associated with the direct effect of growth hormone on the hair tangles.In the later stages of the disease there is loss of hair on the head.
Subcutaneous adipose tissue thickens due to the development of connective tissue.Obesity in acromegaly usually does not happen.
In the initial stages of the disease often marked skeletal muscle hypertrophy and increase muscle strength.In the future, often develop muscle weakness due to degenerative and atrophic changes in the muscles.
In acromegaly occur characteristic changes in the skeleton.Changes skeleton skull above.Disproportionate growth occurs also in other bones.
The vertebrae reinforced periosteal growth occurs in particular on their front surfaces.kyphosis often develops in the thoracic spine and lordosis - the lumbar spine.The ribs thicken and lengthen.The chest becomes barrel-like shape, its volume increases;marked protrusion of costal arches.Thickening of the clavicle and sternum.
thicken the long bones of limbs, but the most characteristic changes occur to the hands and feet (see figure).
Brushes made wide, fingers thicken both by periosteal bone growth and by thickening of soft tissues.Especially noteworthy thickening of the nail phalanges, fingers imparting a characteristic cylindrical shape.Significantly thickened heel bone.Changes in the skeleton clearly identified with the X-ray examination.Often there is an increase in the size of sella and the destruction of its walls.
Besides changes in the shape and size of sella on skull radiographs revealed and other typical signs of acromegaly - an increase in skull size, thickening of the bones of his vault and their external projections, increased pneumatization of the paranasal cavities, particularly the frontal and tapered.Striking also increase and deformation of the mandible (see Fig).
very characteristic radiographic changes are observed in the spine.As a result of bone tumors - especially on the anterior surface of the vertebrae - on lateral spine radiographs indicated disappearance "waist" in the ventral parts of the bodies of the thoracic vertebrae, the existence of dual or multiple loop, reflecting an influx stovanie newly formed bone on the old one.There are also powerful beak projections postvertebralnye arthritis, early ossification of the costal cartilages (see Fig.).
hand and foot bones thickened, most of the phalanx, and especially their head and base;on their side surfaces are sometimes spikes.Marked thickening of the long bones, especially their epiphyses, the structure of which can be rebuilt.The protrusions, bumps, place attachment of muscles and tendons, "stressed."
Often the deformation of joints formed cubitus valgus and genu valgum.
Due to the thickening of cartilage of the larynx and vocal cords voice is low.
Increasing the size of the heart often can be detected only with a special X-ray examination (ortokardiografii), as a result of the simultaneous increase in the volume of the chest percussion boundaries of cardiac dullness in relation to the usual reference points may be normal.In later stages of the disease, due to degenerative changes in the myocardium, heart failure may develop.Data on blood pressure changes in patients with acromegaly are contradictory.One group of researchers observed increase in blood pressure in only one patient with acromegaly of 28, while the other group - 34 patients from 101.
Due chest deformity and reduce ventilation lekih due to a decrease in the mobility of the ribs often develop emphysema,bronchitis.
Increasing the size of the liver rarely detected by palpation, as it remains hidden behind the thickened and elongated ribs.
kidneys are not only increasing in size, but there is an increase of their functions.
blood picture is usually normal, sometimes there is anemia, eosinophilia and monocytosis.
Increased thyroid - diffuse or nodular goiters - are observed in 25% of patients with acromegaly.In view of the fact that 50-70% of patients with elevated basal metabolic rate, it has been suggested a significant frequency have hyperthyroidism.However, histology crop usually does not indicate an increase of hormonal activity of the thyroid gland, the capture of radioactive iodine by the thyroid gland and the level of iodine bound to plasma proteins, the majority of patients is normal.This suggests that in most cases the increase in basal metabolism in acromegaly is due to calorie-gene by direct action of growth hormone.Hyperplasia of the thyroid gland in this case may be related to the stimulating action of growth hormone increase.However, some patients develop hyperthyroidism, which is amenable to conventional antithyroid therapy as well as toxic goiter patients without acromegaly.
- pituitary dwarfism
- clinical picture of acromegaly
- Morgagni syndrome - Stewart - Morel (MORGAGNI - MOREL - STEWART)
- tumors of the anterior pituitary
in later stages of the disease due to the destruction of the anterior pituitary, leading to a decrease in the production of thyroid-stimulating hormone, sometimes develops hypothyroidism.
parathyroid glands increase in size;observed the development of adenomas of the parathyroid glands.Often increased blood calcium.Often, however, and at the same time it increases the level of phosphorus.Men sometimes observed gynecomastia, in women - pathological lactation.
If acromegaly is often a violation of carbohydrate metabolism.Pathological sugar curves indicating decrease tolerance to carbohydrates were observed in 25% of patients 1/3 patients, 27 of 145 patients.Half of patients with impaired glucose metabolism observed is diabetic.Apparently, diabetes mellitus in patients with acromegaly is associated with saharopovyshayuschim action of growth hormone, but occurs only in those patients who have a pre-existing deficiency insular apparatus.There has been and increase in blood insulin activity in patients with acromegaly.Diabetes mellitus in patients with acromegaly is often mild or moderate severity, but there are severe insulin resistance, and form with a tendency to ketosis.In effecting treatment of acromegaly or radiotherapy after removal eosinophilic pituitary adenoma cell diabetes have improved flow up to its full.The emergence of diabetes or worsening of patients with acromegaly is one of the signs of the progression of the underlying disease.
Until recently, the prevailing view that, despite the increase in adrenal weight in patients with acromegaly, their function changes little.Isolation of 17-keto steroids are usually not improved, increase production 17 oxycorticosteroids only observed in individual patients.These clinical observations are consistent with experimental studies that have shown that growth hormone does not affect the quantity and quality of corticosteroids released into the blood by the adrenal cortex and does not alter their sensitivity to ACTH.However, there were reports of an abnormally large increase in the excretion of 17-keto steroids (but not 17 glucocorticoids) in patients with acromegaly after administration of ACTH they observed in patients with acromegaly speed boost of cortisol secretion, and link their lack of symptoms of Cushing accelerated metabolism of cortisol in the hypertrophied liver.
In later stages of the disease may develop gipokortitsizm due to insufficient ACTH production.
In acromegaly usually occurs early in the infringement and lowering of gonadal function in women, which manifests itself in violation of the menstrual cycle.The men at the beginning of the disease sometimes increase libido and potency, often develops in the future impotence.Pregnancy in patients with acromegaly occurs rarely even with the menstrual cycle.In men, sperm count and motility decreased, testicular biopsy revealed hypoplasia of spermatogenic cells.