ACROMEGALIA

From Big Medical Encyclopedia

ACROMEGALIA (Greek akron — an extremity and megas — big) — the neuroendocrinal disease caused by damage of a hypophysis and a hypothalamus and which is shown increase in the sizes of brushes, feet, a facial skeleton, internals and disbolism. It is described in 1886 by the French scientist P. Marie. According to Davydov (L. M of Davidoff), And. occurs quite seldom, more often at women, than at men, and preferential at the age of 18 — 35 years.

An etiology

At most of patients the disease is connected with eosinophilic adenoma of a hypophysis and excess allocation of a growth hormone. However in certain cases changes in a hypophysis are not found, and increase in secretion of somatotropic hormone a front share of a hypophysis is connected, apparently, with strengthening of stimulation of an adenohypophysis somato-tropine-rileasing-hormone of a hypothalamus. The factors promoting development And., are studied insufficiently. In some cases the injury of the head, pathological pregnancy and childbirth, mental injuries and conditions of a stress, acute and hron can provoke strengthening of secretion of a growth hormone. infections (flu, adenoid disease, recurrent quinsies, sypny and typhoid, syphilis, epidemic encephalitis, etc.).

Pathological anatomy

the Tumour of a hypophysis bringing to And., usually eosinophilic adenoma (sometimes a diffusion hyperplasia), is more rare an adenocarcinoma. The chromophobic and mixed adenomas are described (see. Hypophysis, tumors ). Between chromophobic and eosinophilic adenomas there are some distinctions in ultrastructure. It is noted that adenomas with scanty eosinophilic granularity have the greatest hormonal activity.

Expanding out of limits of the Turkish saddle, the tumor can squeeze an optic chiasm. The tumor can be localized in a body of a wedge-shaped bone.

The changes arising owing to hormonal influence of a tumor consist in growth of fabrics, hl. obr. derivatives of a mesenchyma: connecting, cartilaginous, bone, and also parenchyma and stromas of internals (heart, liver, kidneys, spleen, language). On a face increase in a nose, lips, ears, frontal bones is especially noticeable. Reorganization and growth of frontal bones and expansion of frontal sine is noted. Growth of a mandible and its deformation due to change of a corner between a body and its branches («a form of sledge») are followed by disturbances of a bite. Increase in an upper jaw is expressed less. Teeth are moved apart with formation of diastems.

According to A. V. Rusakov, the nature of changes of bones of a skull is not always identical, it is caused by genetic factors: in them are shown in exaggerated, sometimes a grotesque type of line of a structure of a skull, inherent in this individual, e.g. in one cases a dolichocephalia, in others — a brachycephaly. Bones of a skull can be thickened on several centimeters, causing reduction of capacity of a head cavity and a prelum of a brain.

Growth of bones of a skeleton happens due to their reorganization, and the bones keeping cartilaginous departments — and due to resuming of enchondral bone formation. Edges are extended with hl. obr. in front departments that causes lengthening of the front-back size of a thorax. Outside and average zones of costal cartilages are hypertrophied, and internal are exposed to dystrophic changes. Resuming of enchondral growth on border of cartilaginous and bone departments of edges involves formation of acromegalic beads (thickenings in places of joints of edges with a breast).

Vertebrae at And. hl grow. obr. in width. In the field of cartilaginous intervertebral disks enchondral growth is resumed owing to what the backbone is extended quicker, than a breast that leads to strengthening of a chest and cervical kyphosis.

Intervertebral disks hypertrophy and exposed to the dystrophic changes which are followed by ruptures of cartilaginous plates with formation of hernias of Shmorl and the deforming spondylosis.

Tubular bones increase preferential in width by periosteal oppositional growth. The marrowy channel extends at the expense of a rassasyvaniye of a bone and an endosteal surface. Thus, special increase in mass of bones does not happen. Lengthening of bones is more noticeable in brushes and feet that it is connected with resuming of enchondral growth in cartilages of phalanxes.

Bone growths are expressed to hl. obr. in places of an attachment of sinews, fastion, they increase normal tuberosity, combs. It And. differs from hyperostoses, similar to it, at Mari's syndrome — Bambergera (see. Bambergera — Mari a periostosis ).

Microscopic changes of bones are expressed in intensive reorganization of a bone tissue. In a cortical layer the amount of osteons is sharply increased, it is a lot of so-called fragments of osteons. In spongy departments depression of structure, but bone beams are thicker than usual. Changes of joint cartilages are expressed in proliferative and dystrophic processes. Proliferative processes, vozyikayushchy under the influence of the strengthened allocation of a growth hormone, lead to formation of a young cartilage. Physical properties of interstitial substance of such unripe cartilage do not correspond to the mechanical strain experienced by it. Therefore in it dystrophic changes develop. Long alternation of proliferative and dystrophic processes leads to development of the deforming arthrosis which is especially expressed in large joints. In fringe regions of the joint ends of bones there is a resuming of enchondral growth of a bone to formation of regional bone thickenings. In a synovial membrane there can be fleecy outgrowths, sites of cartilaginous and bone fabrics.

And. can be followed by changes of other closed glands: craw, atrophy of the insulyarny device, hyperplasia of strobiloid and goitrous glands, bark of adrenal glands, atrophy of gonads. Cases of a combination of Ampere-second by a parathyroid osteodystrophy, with a pheochromocytoma of an adrenal gland are described. In later stages of a disease atrophic changes of muscles develop.

A pathogeny

It is proved that development And. it is connected with disturbance of regulation of somatotropic function of a hypophysis through the Somatotropinum-rileasing-factor produced by a hypothalamus (see. Hypothalamic neurohormones ), what leads to excess allocation from a hypophysis of a growth hormone (see. Somatotropic hormone ). The increase in somatotropic function of a hypophysis Causing development And., can be at eosinophilic adenoma, an adenocarcinoma and at other tumors of a hypothalamus and a hypophysis, and also at functional disturbances in gipotalamo-pituitary system.

Supersecretion of somatotropic hormone usually causes A. V in adults at whom process of growth of bones is finished children's and youthful age with open epiphyseal regions of growth the increased release of somatotropic hormone causes development of giantism more often.

Clinical picture

Fig. 1. Person sick acromegalia. Fig. 2. Brush and foot of the patient with an acromegalia; on the right — a brush and foot of the healthy person

And. develops gradually; sometimes separate symptoms of a disease appear at early age. The disease is characterized first of all by disproportionate growth and change of a shape of a face (fig. 1). The nose becomes thick, superciliary arches owing to increase in pneumatic cavities considerably act forward; cheekbones are increased, the mandible is given forward, the under lip is blown up. Owing to growth of a mandible between teeth intervals — a so-called diastem are formed. Also growth of ears is noted. The symptom of a macroglossia — big language is very characteristic. Growth of a uvula and voice folds is quite often observed — the voice becomes low. On a skull temporal hillocks and bones of a cranial roof sharply act. The head is considerably increased as at the expense of a thickening of soft tissues of a pilar part of the head that leads to formation of a rough skladchatost on a nape, and due to stratification of a bone tissue.

Changes on distal parts of extremities are very characteristic. Usual type A. — it is the so-called «type en large» (Mari) which is characterized by paliform expansion of brushes and feet, wide fingers (fig. 2) with the acting calcaneus. Hl expand. obr. soft tissues of hands and feet. On phalanxes exostoses are quite often observed. Patients are forced to increase the size of footwear, gloves often. More rare, it is preferential at patients with the unfinished growth of a skeleton, «type en long» when hands and feet are extended in length is observed. The breast, clavicles, edges are excessively developed. On a thorax the cervical and back kyphoscoliosis and a lordosis in lumbar area is quite often observed. Skin at most of patients is thickened, forms folds, usually dry, edematous is more rare. Fibromas, warts are often observed. Head and eyebrows hair thick, rigid, dense. On extremities and a trunk the hypertrichosis is observed; especially sharply it is expressed at women on a face. Vegetation on a body at women is distributed more often on men's type that, apparently, is connected, especially at early stages of a disease, with strengthening of functional activity at these patients of system a hypothalamus — a hypophysis — bark of adrenal glands.

Splanchnomegaly (see) at patients And. meets very often (a cardiomegaly, a hepatomegalia, a splenomegaly, etc.). Not less than in 60 — 80% of cases the atrophy of gonads, an amenorrhea, decrease in sexual desire and sexual weakness (at the beginning of a disease, on the contrary, increase in sexual desire and sexual functions can be observed) is noted. The atrophy of external genitals is not observed, in an onset of the illness even their increase is sometimes noted.

At patients And. there are signs of the increased intracranial pressure: headaches, dizzinesses, vomiting, are more rare epileptoidny attacks.

At many patients And. visual disturbances which are more often a consequence of pressure of a tumor upon a hiazma and optic nerves in this connection for are observed And. the bitemporal hemianopsia (see) beginning usually with an upper external quadrant is characteristic it is frequent in the form of paracentral scotoma. At an asymmetric arrangement of a hiazma unilateral hemianopsias are observed. If increase in a hypophysis happens towards a wedge-shaped bone, then sharp visual disturbances at patients are not noted. Disease it is necessary to make for diagnosis and overseeing to a perimetrium (see). Sometimes the hemianopsia is preceded by achromatopsia therefore it is necessary to make perimetry and for the colors.

From a nervous system pains, especially in spin, an acroparesthesia, the neuralgia connected with pressure of the expanding bones and the deformed vertebrae upon nerve pathways and roots are found. Blood pressure is often increased, apparently, due to activation of function of bark of adrenal glands. In muscles find degenerative and atrophic changes in later stages of a disease what increased fatigue is connected with.

At patients And. carbohydrate metabolism is broken. According to Davydov, at 12% of patients And. the diabetes mellitus, and at 25% — disturbances of a glyukozotolerantnost is noted. Resistant symptom And. it is necessary to consider a polyphagia (see. Bulimia ), less frequent — polydipsia (see), sometimes connected with not diabetes mellitus, and polyuria (see). In peripheral blood at patients And., except a nek-ry lymphocytosis and an eosinophilia, essential deviations is not available. Besides, allocate the high-quality and erased form A. At high-quality And. the vision disorder and function of gonads is not observed. At patients the hl is noted. obr. a hypertrichosis, a hyperhidrosis, the strengthened activity of sebaceous glands leading to appearance of acne on skin, the big language which is badly finding room in an oral cavity. Headaches, an alimentary glycosuria are very characteristic. At the erased form A. (forme fruste) pathological process proceeds very slowly. At this form of a disease large ears, the long and large person are characteristic of patients. They have headaches, preferential at night, sleeplessness, they can have a fibromatosis, a hypertrichosis and some other manifestations characteristic for And.

During puberty coarsening of features and increase in speakers of its parts is sometimes observed; with age these signs gradually smooth out. A passing form is and And. pregnant women, giving sometimes an impetus for development true And. At And. pregnant women the bitemporal hemianopsia is occasionally noted.

Certain authors believe that among healthy people persons meet the akromegaloidny constitution; these are people of high growth, with the large bones, a big jaw acting as superciliary arches, large skulls, a nose, lips and extremities. Under the influence of adverse effects this constitutional form can pass in true And. (cases are described And. after removal of a uterus).

Exists partial (partial) And., striking only language, fingers, foot, etc. These changes can be explained with hypersensitivity of separate fabrics at these individuals to somatotropic hormone of a hypophysis.

Quite often And. it is combined with a thyrotoxicosis, a myxedema, adiposogenital dystrophy and pathology of adrenal glands. At early stages of a disease function of bark of adrenal glands can be increased, and the thyroid gland increases. In late stages of a disease function of these glands can decrease.

Mental disorders. In psychopathological aspect distinguish true And. and akrome-haloid constitution [Bleyler (M. Bleuler)]. The mental disturbances observed at people with the akromegaloidny constitution, the same as at true And., but are considerably less expressed.

And. in itself usually does not cause psychoses. However in most cases at patients characteristic changes of the personality (a peculiar option of an endocrine mental syndrome) develop or, at especially adverse course of a disease, anamnestic - an organic syndrome. Against the background of the mental shifts entering a framework of these syndromes the symptomatic psychoses (see) caused, as a rule, by heavy somatic frustration (increase in intracranial pressure, disturbances of exchange, etc.) can develop.

Psychoses can be hallucinatory paranoid, depressive and hypochiondrial, like acute exogenous reaction. The point of view existing earlier about the special frequency of shizofrenopodobny psychoses and psychoses of type maniac-depressive, and also about communication of Ampere-second schizophrenia and maniac-depressive psychosis at special researches was not confirmed (Bleyler, etc.).

In an endocrine mental syndrome at And. all three components are rather brightly expressed: decrease in mental activity, frustration of mood and frustration of inclinations. Decrease in mental activity is clinically usually shown by apathy, slackness, an aspontannost, lack of an initiative, more rare psychomotor block. The described changes are combined with complacent and euphoric mood and feeling of passive self-satisfaction. Easy euphoria and complacency can be observed at patients even at severe headaches and drowsiness. Unlike euphoria at maniac-depressive psychosis, at patients And. it is not followed by a motive hyperactivity. Are observed as well depressions with alarm, peevishness, tension and tearfulness. Are characteristic for And. causeless mood swings throughout short time. From frustration of inclinations the expressed fluctuations of appetite (periodic sharp strengthening of appetite, emergence of thirst), decrease in a libido, increase in need for a dream and sometimes attacks of drowsiness meet. There can be also periods of the increased physical performance having character of impulsiveness. In the relations with people around patients can show the increased vulnerability, impatience, irritability, an egocentrism.

In the presence of an endocrine mental syndrome of considerable decrease in intelligence does not happen; slowness of mental processes and a nek-swarm decrease in a focus of interest, as a rule, do not prevent such patients to continue work. At the expressed hormonal shifts (increase in products of lactotropic hormone, etc.) peculiar changes of behavior, so-called behavior of mother can be observed (special attention to children, care of them with performance of various maternal duties).

Similar changes of behavior are observed not only at women, but also at men.

If the anamnestic and organic syndrome develops, then it is shown by dysmnesias, decrease in criticism and interests, coarsening of the personality, tactlessness. Outcomes in peculiar autism with gloomy peevishness are described.

See also Endocrine mental syndromes .

Diagnosis and differential diagnosis

Diagnosis And. does not represent special difficulties and it is put on the basis of rather expressed complex of symptoms. Changes of a skeleton at And. are to such an extent characteristic that already by one outward it is possible to make the diagnosis.

From specific laboratory diagnostic testings matter (especially in cases of the erased form A.) researches of content of somatotropic hormone (STG), inorganic phosphorus and not esterified fatty acids in blood. According to M. I. Balabolkin (1966), concentration of somatotropic hormone in blood of patients And. makes 37,51 ± 10,72 mkg of % while normal the level of somatotropic hormone is equal in blood 18,37 ± 6,21 mkg of %. Content of inorganic phosphorus is increased (higher than 4,5 mg of %) at the vast majority of patients; increase in content of not esterified fatty acids is not less often noted (higher than 850 mekv/l).

And. it is necessary to differentiate with Pedzhet's disease at which hl are surprised. obr. a calvaria and a diaphysis of long bones, at And. — a face and fingers (see the Osteosis deforming); with partial giantism, the general giantism (see), Mari's syndrome — Bambergera.

Radiodiagnosis acromegalias promotes specification of the diagnosis. It is recommended to make survey craniographies in side and frontal projections and aim roentgenograms of the Turkish saddle. The side tomogram made through the median sagittal plane of a skull promotes obtaining more exact information on a condition of the Turkish saddle.

In an early phase of a disease when there are no expressed endocrine frustration, there are changes of the Turkish saddle in the form of straightening of a back, thinning of its basis and a sklerozirovaniye of its upper part.

Fig. 3. A skull a sick acromegalia (the roentgenogram in a side projection)

In the expressed cases And. skull of the big size. The facial skeleton (fig. 3) is especially increased. The X-ray pattern of a neurocranium gains diversity in connection with reorganization of the bone drawing with dominance of a hyperostosis and a thickening diploe. Adnexal bosoms, especially frontal increase. The wedge-shaped bosom is narrowed at the expense of a prodavlennost of a bottom of a pituitary pole. The Turkish saddle is deformed: the sagittal and vertical sizes increase several times, the bottom falls, the back deviates, becomes thinner at the basis, but the drawing of a top of a back and adjacent zone amplifies due to consolidation of bone structure. The back inclined shoots are bent kpered; in this regard in a side picture the entrance to a saddle seems narrowed. The contour of a pituitary pole becomes rarefied, the image of a border of a compact layer disappears. It is necessary to consider a possibility of spread of a tumor up or in some party. The last comes to light on thinning and shift in a frontal picture of the front inclined shoot on the party of defeat.

At the direction of growth of adenoma towards visual decussation osteoporosis and supraplacement of one of the front inclined shoots are noted. Only at the considerable size of adenoma can be changed both front inclined a shoot.

Except changes of a skull, in the expressed cases And. in pictures are increased and thickened vertebrae. As a rule, bones of brushes and feet increase. Heads of phalanxes of fingers are usually thickened, nail shoots are expanded, uneven. Long tubular bones are increased, massivna. Hillocks and roughnesses of bones at places of attachments of muscles act more, than normal.

See also Hypophysis , radiodiagnosis.

Forecast depends on the nature of process in a hypophysis. At benign adenoma disease sluggish can also continue decades; if the tumor malignant, a current acute, and death comes at the phenomena of a cachexia. At sharply expressed splanchnomegaly — the forecast adverse. Intercurrent infections, resilience to the Crimea At patients can be a cause of death And. it is lowered.

Treatment

At patients And., at which adenoma radiological is revealed, the medical effect can be reached by destruction of its fabric by means of radiation therapy (see the Hypophysis, radiation therapy of tumors). At increase of vision disorders surgical intervention is shown (see the Hypophysis, surgical treatment).

Pathogenetic treatment And. has the task to block somatotropic function of a hypophysis. It can be reached by means of sex hormones: at men — androgens (Testosteroni propionas on 50 mg intramusculary within a month), at women — estrogen (oestradiol on 1 — 5 mg in a daily dose and in a total dose of 300 — 500 mg). Perhaps also use of the medicamentous funds allocated for an alleged cause of a disease. At use of sex hormones along with clinical improvement at patients normalization of level of somatotropic hormone in blood, and also decrease in concentration of not esterified fatty acids and inorganic phosphorus is observed.


Bibliography: Balabolkin M. I. The maintenance of a growth hormone in blood serum at patients with an acromegalia, Probl. endokrinol. and gormonoter., t. 11, No. 4, page 42, 1965, bibliogr.; Gincherman E. Z. A functional condition of hemadens at an acromegalia, in book: Cerebral and pituitary diseases, under the editorship of E. A. Vasyukova, page 280, M., 1965; Zhukovsky M. A. Children's endocrinology, page 282, M., 1971; The Multivolume guide to internal diseases, under the editorship of E. M. Tareev, t. 7, page 385, L., 1965; Havin I. B. and Gavrilyuk L. I. Akromegaliya and metabolism, Chisinau, 1970, bibliogr.; Tselibeev B. A. Mental disturbances at endocrine diseases, page 12, M., 1966; Bleuler M. Endokrinologische Psychiatric, Stuttgart, 1954; Marie P. Sur deux cas d'acromégalie, hypertrophie singuliere non congénitale des extrémitiés supérieures, inférieures et céphalique, Rev. Méd. (Paris), t. 6, p. 297, 1886; Textbook of endocrinology, ed. by R. H. Williams, Philadelphia — L., 1963.

Pathological anatomy And. — The multivolume guide to pathological anatomy, under the editorship of A. I. Strukov, t. 5, page 276, M, 1959; Remagen W. Neue Befunde bei Akromegalie und nachfolgender Arthrose, Virchows Arch. path. Anat., Bd 340, S. 8, 1965, Bibliogr.; Schelin U. Light and electron microscopical studies on pituitary adenomas in acromegaly, Acta path, microbiol. scand., Suppl. 154, p. 89, 1962.

Radiodiagnosis And. — Kopylov M. B. Bases of radiodiagnosis of diseases of a brain, page 225, M., 1968; Reynberg S.A. Radiodiagnosis of diseases of bones and joints, t. 2, page 62, M., 1964; Fayzulin M. of X. Radiodiagnosis of tumors of a brain, page 56, Kazan, 1967; Bergerhoff W. Die Sella turcica im Röntgenbild, Lpz., I960, Bibliogr.; Dietrich H. Neuro-Röntgendiagnostik des Schadels, Jena, 1954.

E. Z. Gincherman; T. P. Vinogradova (stalemate. An.), D-D-Oryol (psikhiat.), M. of X. Fayzulin (rents.).

Яндекс.Метрика