Hyperplasia of the adrenal glands

Hyperplasia of the adrenal glands refers to serious pathologies, which is explained by the functional characteristics of the paired gland - the production of special hormones (glucocorticoids, androgens, aldosterone, adrenaline and norepinephrine) regulating the vital activity of the whole organism.

[1], [2], [3], [4], [5]

Causes Hyperplasia of the adrenal glands

The causes of hyperplasia of the adrenal glands depend on the type of disease. The emergence of a congenital form of pathology common in clinical practice is preceded by severe functional disorders of the pregnant woman's body.

It should be noted that the causes of adrenal hyperplasia are closely related to stressful conditions, excessive mental stress and strong emotions, increasing the secretion of cortisol (is the main hormone of the group of glucocorticoids).

[6], [7], [8], [9]

Pathogenesis

The phenomenon of hyperplasia is an active increase in cell tissue. The organ that undergoes such changes increases in volume, retaining its original shape. The adrenal glands include the bark and the brain substance. The processes of hyperplasia affect the adrenal cortex more often, and tumors are detected primarily in the brain substance.

As a rule, the disease has an innate character, is inherited or formed as a result of negative external / internal factors. Some diseases are accompanied by the phenomenon of hyperplasia of both adrenals. For example, adrenal hyperplasia is diagnosed in 40% of cases with Cushing's pathology, detected in the middle and old age. The nodular form of hyperplasia is characterized by the presence of one or several nodes, the size of which varies from a couple of millimeters to several centimeters.

[10], [11], [12], [13], [14], [15]

Symptoms Hyperplasia of the adrenal glands

Hyperplasia of the adrenal glands occurs in conditions of metabolic disruptions and with symptomatology, depending on the lack or overabundance of the hormone-glucocorticoid.

Nonclassical forms of hyperplasia are characterized by the following symptoms:

• early hair growth in the pubic region and axillary region;
• excessive and inadequate growth;
• an overabundance of androgens;
• manifestation of terminal hairiness in the body ( hirsutism );
• early closure of growth zones;
• the detection of amenorrhea (no monthly);
• presence of blackheads;
• the bald patches of the region of the temples;
• infertility.

Symptoms of hyperplasia of the adrenal glands are diverse and depend on one or another type of pathology. The most common manifestations of the disease include:

• blood pressure jumps;
• muscular atrophy, numbness;
• development of diabetes;
• increase in body weight, appearance of signs of a "moonlike" face;
• stretch marks;
• osteoporosis;
• changes in the psyche (memory loss, psychosis, etc.);
• disorders of the gastrointestinal tract;
• a decrease in the body's resistance to viruses and bacteria.

Alarming factors are also thirst and the presence of frequent urge to urinate at night.

Nodal hyperplasia of the adrenal gland

About 40% of patients with Cushing's syndrome are prone to bilateral adrenal nodular hyperplasia. Nodules grow to a few centimeters and can be single or multiple. Often nodes are lobed, and pathology is detected more often in old age.

As a consequence of prolonged stimulation of adrenal adrenocorticotropic hormone (ACTH), adrenal nodular hyperplasia affects the formation of an adenoma of an autonomous type. Pathology of the nodular type belongs to the number of hereditary autosomal dominant type of transmission. A clear clinical picture of the formation of nodular hyperplasia has not been established, however, physicians tend to an autoimmune theory of pathogenesis. The severity of the symptoms of the disease grows gradually in accordance with the patient's growing up. In its development, the disease combines signs of an adrenal glandular nature - congenital spotted skin pigmentation (Carney's syndrome), manifestations of mucous neurofibromatosis and atrial myxoma. Among other signs of pathology are noted:

• symptoms of hypertension (headache, dizziness, black "flies" before the eyes);
• dysfunction of conduction and excitation of neurons of muscle structures (convulsive state, weakness, etc.);
• disorders of the kidneys (nocturia, polyuria).

Hyperplasia of the adrenal gland of the nodular type is differentiated according to the stigmas of dysembryogenesis or to small developmental anomalies. These criteria are decisive for establishing the correct diagnosis and represent the difficulty of revealing pathology, since sometimes doctors do not focus on them properly.

[16], [17]

Diffuse adrenal hyperplasia

Hyperplasia of the adrenal glands is divided into diffuse, in which the form of the gland is preserved, and local with the formation of one or more nodules.

It is quite difficult to diagnose diffuse hyperplasia of the adrenal glands by ultrasound, the main methods of recognition of pathology are magnetic resonance imaging and computed tomography. Diffuse hyperplasia may be peculiar to the preservation of the gland's shape with a simultaneous increase in volume. Based on the results of the studies, hypoechoic triangular structures surrounded by fatty tissue are identified. Mixed types of hyperplasia are often diagnosed, namely: diffuse-nodular forms. The clinical course can be washed off or have a marked symptom with a permanent weakness, attacks of panic attacks, increased blood pressure, excessive hair, obesity.

[18], [19], [20], [21], [22]

Nodular adrenal hyperplasia

Two-sided nodular hyperplasia of the adrenal glands (the same nodular) is more common in children and adolescents. Pathology is associated with the phenomenon of hypercorticism and Itenko-Cushing syndrome. The causes of increased production of cortisol lie in the adrenal gland dysfunction or are caused by an overdose of glucocorticoids.

Clinical picture:

• obesity - uneven type, fatty tissue is deposited mainly in the neck, abdomen, chest, face (hence the "moon-like" oval face, "climacteric" hump);
• muscular atrophy - pronounced on the legs and shoulders;
• dry, thinned skin, marble and vascular pattern, purple or violet striae, areas of hyperpigmentation;
• development of osteoporosis - thoracic and lumbar division, compression-type fractures in combination with the strongest pain syndrome;
• the appearance of heart failure and heart rhythm disturbance;
• changes on the part of the nervous system - a depressive state along with retardation or, in contrast, complete euphoria;
• presence of diabetes mellitus;
• excessive hair in women in the male type and the formation of amenorrhea.

Hyperplasia of the adrenal gland of the nodular type has a favorable prognosis for early diagnosis and treatment.

Micronodular hyperplasia of the adrenal gland

Local or nodular forms of hyperplasia are divided into micro- and macronodular pathologies. Micronodular hyperplasia of the adrenal gland develops against the background of the active action of adrenocorticotropic hormone on the cells of the gland with the subsequent development of adenoma. The adrenal gland produces an increased amount of cortisol, and the pathology itself is referred to the hormone-dependent form of Cushing's disease.

Hyperplasia of medial adrenal gland

As practice shows, information about the adrenal glands is based on the morphological (postmortem) parameters. Based on the data of medical research, in which about 500 bodies of healthy people were examined during life (from 20 to 60 years), it is possible to judge the state of the adrenal glands. The paper presents data on the shape and size of the glands due to the axial and frontal sections (the thickness of the cut is 5-7 mm), which makes it possible to obtain the height of the medial stem of the adrenal glands, as well as the length of the lateral stem.

Based on the results of the morphological study of the adrenal gland, it was concluded that the adrenal glands with abnormalities without a small-node or diffuse hyperplasia refer to adenopathy. In turn, adenopathy is understood as a condition of the adrenal gland, in which during a time and under the influence of a number of factors, hyperplasia will form or the initial stage of the disease will be stopped (for example, as a result of treatment) and the gland will return to normal functioning. It should be noted that deviations in the size of the adrenal glands, which include hyperplasia of the medial leg of the adrenal gland, were found in 300 people.

Hyperplasia of the adrenal cortex

Adrenogenital syndrome refers to congenital hyperplasia of the adrenal cortex due to the dysfunction of the activity of enzymes responsible for steroid biosynthesis. These enzymes regulate the hormones of the adrenals and gonads, so it is possible to simultaneously break down the hormonal secretion of the genital area.

Hyperplasia of the adrenal cortex of an innate nature is associated with various gene mutations leading to a disruption of the synthesis of cortisol. The disease develops against the background of a decrease in the level of cortisol, an increase in the parameters of ACTH in the blood and the appearance of bilateral hyperplasia.

Pathology is recognized by the following distinctive features:

• predominance of masculine traits on the background of hormonal failure;
• excessive pigmentation of the external genital area;
• early hair growth in the pubic area and under the arms;
• acne;
• later onset of the first menstruation.

It is necessary to differentiate the hyperplasia and tumor of the adrenal glands. To this end, hormonal diagnosis is performed - laboratory tests of urine and blood to detect hormone levels.

Adrenal Hyperplasia in Adults

Hyperplasia of the adrenal gland is more often congenital in nature and is detected in small children, which allows you to start hormonal therapy as early as possible. The erroneous identification of the sex at birth, as well as the lack of timely treatment, often leads to various psychological difficulties experienced by patients in connection with the development of secondary sexual characteristics.

Treatment of adult women may be necessary for feminization, and men to eliminate sterility, when testicles are atrophic and spermatogenesis is absent. The appointment of cortisone to older women helps to eliminate external signs of hyperplasia: the shape of the body changes as a result of the redistribution of fatty tissue, facial features become feminine, an acne occurs, and the mammary glands grow.

Hyperplasia of the adrenal glands in adult patients requires constant maintenance doses of drugs. With constant monitoring of the state, cases of ovulation, pregnancy and the birth of healthy children are described. Initiation of therapy for virilizing symptoms at the age of 30 years, the ovulatory cycle may not be established, it is often found that uterine bleeding is not associated with the cycle. In this case, appoint estrogens and progesterone.

[23], [24], [25]

Forms

Hyperplasia of the adrenal glands is divided into:

• hypertensive;
• viril;
• salting.

The viril subtype is associated with the secretory activity of androgens, as a result of which there is an increase in the external genitalia, as well as excessive and early appearance of hair, acne, and rapid development of the musculature. The hypertonic form is manifested with the increased action of androgens and mineralocorticoids, which negatively affects the vessels of the fundus, the kidneys and causes hypertensive syndrome. Soltering hyperplasia is caused by increased production of androgens against the background of the absence of other hormones of the adrenal cortex. This type of pathology provokes hypoglycemia and hyperkalemia, threatening dehydration, weight loss and vomiting.

Hyperplasia of the left adrenal gland

The adrenal gland on the left has the form of a crescent, its upper anterior surface is limited by the peritoneum. Hyperplasia of the gland tissue refers to the functionally active tumors (often benign) and causes endocrine disorders.

Modern medicine has opened a mechanism for the formation of pathology at the cellular and molecular level. There are facts that hyperplasia of the left adrenal gland and production of hormones are interrelated with changes in the conditions of intercellular interaction (the presence of defects in the gene and chromosome sites, the presence of a hybrid gene or chromosomal marker). The disease can be both hormone dependent and not dependent.

The indication for surgical removal is the detection of overgrowth over 3 cm. The retroperitoneal resection is performed by the method of laparoscopy, which allows to minimize the postoperative period. Neoplasms of lesser magnitude are observed to assess the tendency of the spread of the focus of hyperplasia. In addition to laparoscopic intervention, it is possible to use lumbotomy access according to Fedorov on the left.

[26], [27], [28]

Diffuse hyperplasia of the left adrenal gland

Diffuse hyperplasia of the left adrenal is diagnosed in most cases of arterial hypertension. The condition is often reinforced by headaches, myocardial dysfunctions, eye fundus pathologies. Cardiac symptoms are due to sodium retention, hypervolemia, narrowing of vessels and an increase in resistance along the periphery, activation of the receptors of the vascular bed to pressor effects.

In the patient's condition, muscle weakness, presence of convulsions, dystrophic changes in the muscular and nervous structures are also noted. Often there is a "renal syndrome", manifested by an alkaline reaction of urine, nicturia, a strong thirst.

Diffuse hyperplasia of the left adrenal is differentiated by computer or magnetic resonance imaging. Through these survey methods, it is possible to detect changes in the gland with a certainty of 70 to 98%. The purpose of selective phlebography is to determine the functional activity of the adrenal gland with obtaining data on the amount of cortisol and aldosterone in the blood.

Diffuse and diffusive-node hyperplasia of the cortex is associated with a significant increase in the activity of the adrenal gland. Conservative therapy in this case gives weak results, so one-sided adrenalectomy is recommended. Simultaneous presence of diffuse hyperplasia and aldosterome has the most unfavorable outcome even in the case of surgical intervention.

Nodular hyperplasia of the left adrenal gland

The phenomenon of primary hyperaldosteronism is directly associated with increased arterial pressure, serving as an important clinical sign of excess aldosterone in the cortical layer of the adrenal gland. Variants of the course of the disease: diffuse or diffusive-nodal hyperplasia of the left adrenal / right adrenal (can be bilateral) in the presence / absence of an adenoma of a secondary type. Symptoms include cardiovascular (pressure jumps, hearing impairment, etc.), muscle (weakness, atrophy), renal (nocturia, polyuria, etc.) and nerve dysfunctions (eg, panic attacks).

As a result, CT or MRI is rounded in shape, a hypoechoic formation that is easily taken for an adenoma. Based on the results of the analyzes, an increase in the production of cortisol, aldosterone and renin in the blood is confirmed. Daily urine tests reveal overestimated values of 17-CS and 17-ACS. Externally observed - increased hair growth, overweight, the presence of stretch marks on the body.

Nodular hyperplasia of the left adrenal is treatable by surgical methods, followed by maintaining a stable hormone state containing medications.

Nodular hyperplasia of the left adrenal gland

The concepts of "family pathology of Itenko-Cushing", "family Cushing's syndrome with primary adrenocortical adenomatosis", "primary adrenocortical nodular hyperplasia", "ACTH-inactive Cushing's disease", etc. Widely distributed in clinical practice. Under this set of terminology is meant nodular hyperplasia of the left adrenal or right adrenal. In most cases, pathology carries the hereditary nature of the autosomal dominant mode of transmission. Formation of nodular hyperplasia is argued by autoimmune theory. A feature of the disease is the functional isolation of the cortical layer of the adrenal gland, which is detected in the blood by examining the level of cortisol and ACTH or the presence of 17-ACS in urine.

Nodular hyperplasia of the left adrenal gland, described in a number of studies, is determined by the signs of a cushingoid syndrome of a manifest or expanded clinical picture. Most often, the disease develops secretly with a gradual increase in symptoms, depending on the age of the patient. Nodular hyperplasia is characterized by manifestations of the adenopocellular origin, which include pigmented spots on the skin, the formation of tumor processes of various localization and neurological symptoms.

[29], [30], [31], [32], [33]

Hyperplasia of the right adrenal gland

The adrenal gland on the right resembles a triangle in shape, the peritoneum adjoins its lower part. Hyperplasia of the gland is a fairly common disease, often detected at a neglected stage or after the patient's death. The difficulty of differentiating pathology, if the disease is not hereditary, is caused by the asymptomatic course of pathology. To reveal the tumor at the beginning of development is possible due to ultrasound, MRI or CT. Manifestations of clinical symptoms of the Itenko-Cushing's disease are often supported by echoscopy data with the definition of an echopositive neoplasm on top of the right kidney. To finalize the diagnosis of hyperplasia of the right adrenal, laboratory tests of blood and urine are carried out.

Hyperplasia is diffuse or focal. The latter form is subdivided into a macro- and a micro-node, which, when examined by means of ultrasound, does not differ from the tumor processes of the gland. The symptomatology of the disease is diverse for each specific case, including arterial hypertension, diabetes mellitus, muscle weakness, changes in kidney function, etc. The clinical picture is characterized by a blurred and crispy character. Proceeding from the severity of the course of hyperplasia, the patient's age and individual characteristics, a therapeutic tactic is developed, often involving operational impact.

Nodular hyperplasia of the right adrenal gland

With Cushing's syndrome, in almost 50% of clinical practice, nodular hyperplasia of the right adrenal gland or the left adrenal gland is observed. A similar disease is diagnosed in patients of the middle and old age group. The disease is accompanied by the formation of several or one node, the magnitude of which varies from a couple of millimeters to an impressive size in centimeters. The structure of the nodes is shared, and in the space between the nodules the hyperplasia focus is located.

The disease is differentiated according to external signs: obesity, thinning of the skin, muscle weakness, osteoporosis, diabetes of a steroid type, reduction of chlorine and potassium in the blood, red striae on the hips, abdomen and chest. Pathology can develop secretly without significant clinical symptoms, which greatly complicates the task of a diagnostic doctor. For the purpose of classification of pathology, laboratory analyzes of blood and urine, CT and MRI studies, histological tests are used.

Treatment of adrenal hyperplasia on the right is based on diagnostic data and the type of disease. In most cases, surgical resection is shown, which allows to normalize blood pressure and return the patient to a full life.

Congenital adrenal hyperplasia

Hyperplasia of the congenital type is classified according to the classical and nonclassical course. The classical manifestations of the disease include:

• lipoid form of pathology is a fairly rare disease associated with a deficiency of the enzyme 20.22 desmolase and a deficiency of steroid hormones. In case of survival, the child develops severe adrenal insufficiency and inhibition of sexual development;
• Congenital adrenal hyperplasia due to a lack of 3β-hydroxysteroid dehydrogenase with severe salt loss. In this case, girls due to active development of male sex hormones during the period of intrauterine development sometimes reveal external genital organs, formed in accordance with the male type. Boys can develop according to a female phenotype or have failures of sexual differentiation;
• diffuse subtype of hyperplasia (lack of 17α-hydroxylase) - is very rarely diagnosed. Pathologies are characterized by clinical manifestations of deficiency of glucocorticoids and hormones of the sexual sphere. Toddlers suffer from low blood pressure and hypokalemia associated with the lack of a sufficient number of potassium ions. For girls, the disease is characterized by a delay in puberty, and for boys - signs of pseudohermaphroditism;
• diffuse type of hyperplasia with a lack of 21-hydroxylase refers to simple virilizing forms.

[34], [35], [36], [37]

Diagnostics Hyperplasia of the adrenal glands

Diagnostic measures consist in clinical examination, laboratory studies (giving clinical, hormonal and biochemical picture), instrumental and pathomorphological examination. Methods of research to determine the functional indexes of the adrenal glands are obtaining information about the concentration of hormones and their metabolites in urine and blood, and also performing certain functional testing.

Laboratory diagnostics of adrenal hyperplasia involves two methods - enzyme immunoassay (ELISA) and radioimmunoassay (RIA). In the first case, the amount of hormones in the blood serum is detected, and in the second case, the presence of free cortisol in urine and cortisol in the blood. RIA by studying the blood plasma allows you to determine the number of aldosterone and the presence of renin. The inclusions of 11-oxycorticosteroids give information on the glucocorticoid function of the adrenal glands. It is possible to evaluate the functioning of the androgenic and partially glucocorticoid component by releasing free dehydroepiandrosterone in the urine. As for functional tests, dexamethasone samples are used that help differentiate hyperplasia or adrenal tumor processes from similar in clinical signs.

Hyperplasia of the adrenal glands is examined by X-ray methods: tomography, aorto- and angiography. The most modern diagnostic methods include: ultrasound, computer and magnetic resonance imaging, radionuclide scanning, giving an idea of the size and form of the adrenal gland. In some situations, an aspiration puncture performed by a thin needle under ultrasound and computed tomography together with a cytological examination may be necessary.

[38], [39], [40], [41], [42], [43],

Differential diagnosis

Adrenal hyperplasia is differentiated immediately after birth or in the first years of life, more often pathology is found in female infants. An important role is assigned to the early diagnosis of the disease, since the neglected process most unfavorably affects all body systems - digestive, nervous, vascular, and the like.

Treatment Hyperplasia of the adrenal glands

Therapeutic tactics for the most part boils down to the construction of a hormone regimen. It is not possible to prove the advantage of a specific program for the administration of substances or their combinations. In general, glucocorticoids - hydrocortisone, dexamethasone, prednisolone, cortisone acetate and various combinations of drugs are prescribed. And the treatment of adrenal hyperplasia is possible with two or three equal doses of hormonal drugs per day, as well as a general dosage in the morning or afternoon. For children with salt deficiency syndrome, mineralocorticoids are recommended and a simultaneous increase in daily salt intake up to 1-3 g. In order to stimulate the formation of secondary sexual characteristics, adolescent girls are prescribed estrogens, and boys androgens.

Hyperplasia of the adrenal gland requires a surgical procedure. Operations are indicated when an external genitalia of an intermediate kind is identified. Correction of sexual characteristics in accordance with the genetic sex is desirable to be carried out in the first year of life of a small patient, provided the child's condition is stable.

Treatment of nodular hyperplasia of the adrenal gland

The main treatment of nodular hyperplasia of the adrenal gland is the surgical removal of the affected adrenal gland. Operations with the preservation of the altered adrenal (resection, enucleation, etc.) are recognized as ineffective in view of frequent relapses.

Among modern minimally invasive surgical methods, laparoscopic adrenalectomy is distinguished. Endoscopic technique is safe and practical. Particular attention deserves extraperitoneal adrenalectomy, on the one hand requiring more skill from the surgeon, and on the other - better tolerated by patients. After such an operation, an extract from the hospital is possible in a few days and in a few weeks the patient returns to a full life. Among the advantages of laparoscopy - the absence of scar surface, the weakening of the muscular corset of the lumbar region.

Removal of the adrenal gland leads to an increase in the load on the remaining healthy organ, which makes it necessary to introduce hormone replacement therapy for the entire period of life. Appropriate treatment appoints, if necessary corrects and constantly supervises the doctor-endocrinologist. After adrenalectomy, one should minimize physical and mental effects, forget about alcohol and hypnotics.

Treatment of congenital hyperplasia of the adrenal cortex

Congenital hyperplasia of the adrenal virilizing type is treatable with cortisol, cortisone or similar substances of synthetic origin. Therapy is conducted with a constant monitoring of the daily content of 17-ketosteroids in accordance with the age norm.

Often, the treatment of congenital hyperplasia of the adrenal cortex is performed by intramuscular injections of cortisone. The initial dosage is chosen to suppress adrenocorticotropic function of the pituitary:

• children under 2 years old - 25 mg / day;
• older children and adults - 50-100 mg / day.

The duration of therapy varies from 5 to 10 days, after which the amount of cortisone administered is reduced to a supporting function. A variant is possible when the dosage remains the same, but the frequency of the injections changes (every 3-4 days).

Oral use of cortisone is divided into daily portions for reception 3-4 times. To achieve the desired effect, it will take two or even four times more tablet substance compared to a liquid solution for injection.

Babies are shown cortisone, while older children and adult patients are successfully treated with prednisolone. The primary daily dose, which reduces the production of 17-ketosteroids up to permissible values, is 20 mg. Approximately in a week the amount of the drug is reduced to 7-12 mg / day.

The most active glucocorticoid drugs, such as dexamethasone and triamcinolone, not only do not have advantages over traditional therapy, but also have severe side effects - psychosis, hypertrichosis, manifestations of hypercorticism, and the like.

Prevention

Hyperplasia of the adrenal glands of any kind in a family history is a predisposing factor for the reference to a geneticist. A number of congenital forms of the pathology of the adrenal cortex are revealed by prenatal diagnosis. Medical conclusion is established during the first three months of gestation based on the results of a chorion biopsy. In the second half of pregnancy, the diagnosis is confirmed by examining the amniotic fluid at the level of hormones, for example, the indicators of 17-hydroxyprogesterone.

Prophylaxis of adrenal hyperplasia is regular examinations, including screening testing of newborns, which allows to establish a congenital form of hyperplasia based on the study of capillary blood taken from the toe of a baby.

So, in most cases, preventive activities concern only future parents who must:

• consciously approaching issues of pregnancy planning;
• undergo a thorough examination for possible infectious diseases;
• Eliminate threatening factors - the effect of toxic substances and radiation;
• visit genetics, if earlier in the family hyperplasia of the adrenal gland was detected.

[44], [45], [46], [47], [48]

Forecast

Hyperplastic changes are observed more often on both adrenal glands. The increase in the gland in the volume leads to an increase in the level of hormones.

Hyperplasia of the adrenal glands provokes certain diseases:

• pathology Itenko-Cushing - visually diagnosed in obesity with an increase in the upper body and swelling of the face ("lunar"), with the muscular structure and dermis atrophy. The syndrome is characterized by hyperpigmentation, the appearance of acne and strong hair. In addition to the fact that there are problems with healing of the injured skin, there are violations in the support system, jumps in blood pressure are observed, disorders of the sexual and nervous spheres are revealed;
• Cone's disease - apparently does not manifest itself in any way, is connected with the withdrawal of potassium from the body and the accumulation of sodium. As a result, fluid accumulates, which increases the risk of stroke, causes a decrease in sensitivity, leads to convulsions and numbness of the limbs.

Hyperplasia in men is treated with medicines before the end of puberty, hormonal therapy of female patients is carried out throughout life. Women with hyperplasia of the adrenal gland are shown to undergo a regular examination, mandatory pregnancy planning and control of labor.

In adulthood, the prognosis of adrenal hyperplasia is favorable in the case of radical surgical treatment. In some cases, there is enough permanent medical control and regular hormone therapy that stabilizes the patient's condition, improving the quality of life.

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