Adrenal insufficiency in children symptoms. How to check the adrenal glands and what tests should be taken? Clinical aspects of Addison's disease - video
The adrenal glands belong to the endocrine system and regulate metabolic processes in the body. These bodies take important role in puberty, maintaining the balance of salts and water. Also support vegetative functions active, participate in biochemical reactions. They play one of the main roles in correct formation intrauterine fetus and healthy development of the child.
Anatomical and physiological features and functions of the adrenal glands in children
The adrenal glands have an outer cortical layer and an inner medulla. The function of the adrenal cortex is to process biologically active substances(steroids). Their synthesis with cholesterol produces hormones that regulate the body's metabolism. This includes cardiac glycosides - substances that regulate the activity of the heart. The adrenal glands are able to synthesize complex hormonal substances that have no analogues in nature.
The first 10 days after birth, babies are at risk of developing adrenal insufficiency. A child from the first day of birth to 10 days has a threat of adrenal insufficiency. After this period, the adrenal glands continue to work and develop normally. During the period child development these small organs undergo several stages of change in mass and percentage active substances in the composition. The final formation of the anatomical and physiological features of the adrenal glands ends by about 20 years.
Pathologies and diseases
congenital agenesis, fusion
Agenesis (lack of development) of the adrenal glands almost never occurs, if it does, then the outcome is fatal. Less often, one organ is poorly developed and during life this is not particularly manifested, since another takes over its function. In some cases, fusion (fusion) of the adrenal glands occurs in front of or behind the aorta. Sometimes the adrenal glands are fused with nearby organs - the liver and kidneys. With malformations of the abdominal wall, there is a splicing of them with the lungs. Sometimes there may be an abnormal location of the adrenal glands, not in its usual place.
Adrenoleukodystrophy is a hereditary disease that affects the nervous system and adrenal glands. In the form of a hereditary disease, adrenoleukodystrophy is also isolated. As a result, brain nerve cells are damaged at the membrane level. The body is unable to break down long chain fatty acids. As a result, the nervous system and adrenal glands are affected. Men are more likely to get sick different ages and the carriers are women. In children from the age of 4, the disease develops rapidly. If such adrenal insufficiency is not detected at an early stage, then a lethal outcome is likely in 5-10 years.
Hemorrhage
Adrenal hemorrhage occurs in premature infants with insufficient vascular density due to birth trauma, as well as infections, sepsis. Hemorrhage and partial necrosis of tissues lead to the formation of cysts or calcified deposits. If the hemorrhages are extensive and bilateral, then usually everything ends in death. This is due to the developing acute adrenal insufficiency.
Adrenal insufficiency develops when there is a lack of adrenal hormones. If the adrenal glands sharply reduce the production of hormones or stop their synthesis, acute adrenal insufficiency occurs. Sodium ions and chlorides are lost, the intestines cannot absorb them in full. The body loses fluid, the blood thickens, the violation of circulation ends in shock, the ability of the kidneys to filter decreases. When hormones are produced with a constant deficiency, chronic adrenal hypofunction is formed, it is divided into 3 types:
Congenital adrenal hyperplasia
Congenital disorders of the adrenal glands lead to several clinical pictures. As a result, a decrease in the work of the adrenal cortex blocks the protein and hyperplasia occurs (an increase in the internal number of cells), along with this, the organ grows in volume. The strongest (complete) protein blockade occurs in 3 out of 4 cases, manifests itself immediately after birth. Adrenal insufficiency is formed, androgens (types steroid hormones) are in excess. This leads to the sexual development of girls in the male pattern, and boys are formed too quickly and have pronounced sexual characteristics from an early age. Other forms have slight manifestation and do not lead to adrenal insufficiency in children. The protein is partially blocked. As a result, girls experience hidden changes in the form of infertility. Very slight violations may not appear at all throughout life.
Primary hyperaldosteronism
The disease is characterized by excessive production of the hormone aldosterone. Hyperaldosteronism is a disorder of the adrenal glands that results in the production of too much aldosterone. This substance retains sodium and is responsible for the excretion of potassium by the kidneys. Renin regulates blood pressure blood vessels causing them to shrink. This enzyme also regulates sodium and potassium metabolism. An excess of aldosterone produced inhibits the action of renin, which causes an increase in blood pressure, deficiency of magnesium and potassium. Research in this area was conducted by the American scientist Jerome Conn and the pathology is called "Conn's syndrome" or "primary hyperaldosteronism."
Potassium deficiency leads to fatigue muscles, heart attacks, headaches and seizures. The level of calcium also decreases, which leads to numbness of the limbs, cramps, and a feeling of suffocation. Taking potassium and calcium supplements is not prescribed, since there is enough of them in the blood, just unhealthy adrenal glands do not allow them to be well absorbed. Tumors grow in the outer (cortical) layer and in the inner (brain). Are removed surgically supportive treatment is given. An increase in even benign tumors can provoke diabetes mellitus, kidney dysfunction, and a violation of the reproductive system. As a result of the progression of a tumor with metastases, secondary neoplasms occur. The most common among malignant tumors adrenals in children. Its occurrence is assumed due to heredity, the influence of other factors has not yet been determined. The main percentage of the disease occurs in children under the age of 5 years. The third part of cases falls on babies up to a year. And only a small percentage are diagnosed before the age of 10.
Adrenal insufficiency in children is a disease that occurs when there is insufficient secretion of a certain amount of hormones by the adrenal cortex. The disease has no specific manifestations and can be either acquired or congenital form. The adrenal glands, like all organs, are subject to unfavorable factors, them normal work depends on good circulation.
Responsible for the release of hormonal secretion and its entry into the bloodstream hormonal glands. Our body is subject to many processes that occur due to proper circulation and required amount hormones in our body. An important role is given to the participation of hormones in the metabolic process. Incorrect work of our body is associated with an increase or decrease in hormones.
The threat of the onset of the disease occurs in the first months of the baby's life, later the work of the adrenal glands normalizes, and they continue to function normally. In the process of development of the child's body, a number of functional changes are observed in these organs. At the age of 20, complete formation occurs.
Anatomical and physiological features and functions of the adrenal glands in children
In the structure of small-sized organs, it is worth noting the presence of two different layers. Each layer performs certain functions. They are:
- Outer cortical layer;
- Medulla.
The outer layer is called the cortex, and the inner layer is medulla. The structure of the medulla involves nerve and glandular cells, as well as nerve fibers. The medulla plays a secondary role, in contrast to the cortical one. Removal of the medulla does not pose a threat to the life of the child.
The function of the cortical substance is to process steroids and is responsible for the production of hormones, the main task of which is the regulation of the process of the substances of the whole organism. In a newborn child, the mass of one adrenal gland is equal to seven grams, and the value is slightly smaller size kidneys. It is customary to separate the two adrenal cortices. The cortex, in turn, has two zones:
- Fetal;
- Definitive.
During the primary formation of the adrenal glands undergo a number of functional changes. In the first month of a child's life, their mass decreases by half, changes in the cortical zone are significant. At the age of about one year, the main role is assigned to the definitive zone, which performs the main functions in the child's body.
In this period, the appearance of glomerular, fascicular and reticular zones is noted. The cortical part is formed in a child up to 11-14. The medulla increases in size. The end of its growth falls on the period of 10-12 years. An increase in the body occurs by the age of 20. By this period, their work can be compared with the work of the adrenal glands of an adult.
Symptoms of the disease in children include:
- muscle weakness;
- Rapid fatigue;
- Cardiopalmus.
The manifestation of weakness, loss of appetite, nervous overexcitation are the causes of many diseases and require diagnosis, but many parents are wondering if the child has. In this case, doctors recommend taking tests for the amount of hormones. The test results will reliably confirm the existence of a certain number of hormones in the child's blood. According to their results, you can draw the appropriate conclusions.
Pathologies and diseases
Neuroblastoma is a type of malignant tumor. it serious disease causes damage to the kidney tissue. The emergence of this kind malignant neoplasm very dangerous and can lead to the development of metastases, which in turn affect the lymph nodes, internal organs, cells skin. As a rule, the tumor does not manifest itself in early age, it can be accidentally discovered at a pediatrician's appointment or during ultrasound.
The presence of neuroblastoma of the right and left adrenal gland in children is characterized by rapid growth and development. Neuroblastoma of the adrenal gland in children requires surgical intervention. The detection of neuroblastoma early stages development is the key to the successful recovery of a small patient.
Adrenal damage and nervous system occurs with sympathoblastoma. This type cancer can develop in embryonic period, during the formation of the organs of the nervous system in the embryo.
With congenital adrenal cortex, there are changes in hormonal level. For example, in boys there is an increase in the penis, darkening of the skin in the scrotum, breaking the voice. In girls, development occurs in a male pattern. Enlargement of the clitoris, lack of menstruation, fusion of the labia. These signs indicate functional disorders in the child's body.
Autoimmune destruction of the adrenal cortex is observed in most cases and is one of the causes of primary chronic adrenal insufficiency. The manifestation of autoimmune destruction of the cortex is due to the presence of antibodies in the child's blood.
Congenital agenesis (fusion)
Cases of congenital agenesis in newborns are rare. However, if the presence of congenital agenesis in a child is diagnosed, the consequences can be the most severe, up to death. congenital agenesis is a disease in which there is no development of one of the organs, in this case, all the functions in the body are performed by another. Perhaps the fusion of two organs with the liver, kidneys, which negatively affects the work of the whole organism.
Adrenoleukodystrophy has a congenital form, the disease is inherited. As a result of the disease, the adrenal glands and the nervous system are affected. The development of the disease is progressive. If such a disease is not identified in a baby at the age of 4 years, a fatal outcome is possible by the age of five or six. Timely contact with a specialist will help to avoid the kids of the disease.
Hemorrhage
The cause of hemorrhage in the adrenal glands may be the presence of an infection in the child's body. More often, hemorrhage in the adrenal glands in newborns is expressed in insufficiently formed vascular density. The appearance of neoplasms (cysts, tumors) was noted. The presence of infection, stressful situations can cause bleeding. At congenital diseases adrenal glands in newborns, a lethal outcome is often noted.
Hemorrhage in the adrenal glands in children is a severe form of the disease. The cause of hemorrhage can be:
- birth trauma;
- Infection;
- Sepsis.
Hemorrhages are:
- Central character;
- focality;
- Expressed in the form of heart attacks.
Hemorrhage in the adrenal glands in children can have the most severe consequences. The most severe pathology is hemorrhage in the adrenal glands, in newborns it often leads to death.
Adrenal insufficiency
A deficiency in the presence of hormones in the blood of a baby causes adrenal insufficiency. Inadequate secretion of hormones is the cause of acute adrenal insufficiency in children. A deficiency in hormone production causes the kidneys to malfunction, forming chronic hypofunction. Clinical symptoms chronic insufficiency associated with the destruction of the adrenal glands, i.e. their improper functioning.
Congenital adrenal hyperplasia
A congenital disorder of the cortex leads to adrenal problems in children. At wrong work, namely, as a result of a decrease in the produced protein, hyperplasia occurs. As a result, the child suffers from adrenal insufficiency. There is a hormonal failure, which leads to improper functioning of the body as a whole. Acquired dysfunction of the adrenal cortex also occurs. There are symptoms of the disease congenital hypoplasia. A pediatrician is able to immediately diagnose disorders in the child's body. In girls, development is observed according to the male type; in boys, maturation is noted earlier.
Primary hyperaldosteronism
Cause this disease is the production of a hormone called aldosterone. The hormone is produced in the body in large quantities. The task of aldosterone is to excrete potassium by the kidneys. With excessive secretion of the hormone, there is a violation of the enzyme renin, which is responsible for balanced work vascular system and blood pressure. A sign of hormonal imbalance leads to the appearance of cardiac pathologies, headaches and convulsions at night. High blood pressure can be a sign of a hormonal imbalance.
Neoplasms
Often the adrenal glands are affected by benign tumors. Damage frequency benign tumor higher than malignant. The tumor affects the adrenal cortex, and tends to grow. Like any neoplasm, they require surgical intervention and subsequent medical therapy. Many neoplasms in children do not have symptoms of the disease.
Neuroblastoma of the adrenal gland in children is a type of malignant tumor. Early detection of neoplasm successful treatment. There have been cases of hematomas in newborns. A hematoma in a newborn baby can be the result of a birth injury, or an acquired one. You can determine the hematoma by touch, it will not be superfluous to pass ultrasound examination.
Adrenal cysts
Adrenal cysts are rare in childhood. The disease does not manifest itself in the early stages of development. However, this disease of the adrenal glands in children can progress. What is expressed in an increase in the volume of the cyst in the child. You can determine the cyst in a baby during an ultrasound examination.
Why are such deviations dangerous?
The adrenal glands play an important role in the life process. They are assigned the main function - the full physical development of the body. If these organs do not work properly, the nervous, the cardiovascular system. The metabolism in the body is due to their proper functioning, in adolescents this is expressed in the production of sex hormones. Hormonal imbalances may cause various kinds diseases.
In medical practice the term syndrome is present chronic fatigue. The adrenal glands are responsible for the nervous system, so stressful situations have negative impact on their full time job. A nervous system disorder can cause adrenal fatigue.
Diagnostics
In order to put correct diagnosis, you should consult a specialist and go through laboratory research. First of all, take tests, undergo an ultrasound examination for the appearance of neoplasms (tumors, cysts). Exclude the appearance of malignant tumors, such as neuroblastoma of the adrenal gland in children, as well as the presence of other malignant tumors. Take a blood test to establish the norm of cortisol, a hormone produced by the adrenal cortex. Hyperfunction of the adrenal cortex occurs with a decrease in the level of cortisol in the blood plasma.
Cortisol affects the metabolism of proteins, fats and carbohydrates. About ten percent of cortisol is in the blood plasma in a non-protein-bound state. Cortisol is produced by the adrenal cortex and serves to keep the heart and nervous system stable. A situation in which the level of cortisol in the blood plasma is less than the established norm leads to improper functioning of the adrenal glands and the nervous system. The norm of cortisol in adults is 10 mg per deciliter. It is possible to increase the level of cortisol in the blood or decrease it, so it is worth taking tests twice a day.
Treatment
Adrenal insufficiency in children today is an urgent problem. Treatment is prescribed depending on the severity of the disease and general condition patient. In order to restore the broken hormonal balance, appoint drug treatment using hormone therapy. In this case, drugs such as Medrol, Glucocorticoid and Cortef are used.
When diagnosing primary adrenal insufficiency, treatment is carried out with glucocorticoids. Glucocorticoids are involved in the metabolic process. Therefore, the growth of glucocorticoids affects the proper metabolism in the body and its balanced work. Hydrocortisone is prescribed for replacement therapy. Replacement therapy with glucocorticoids should be carried out with caution.
A sudden increase in dose may result in side effects. Therapy with gluco drugs includes the use of glucocorticoid drugs, one of which is prednisolone. The drug has an anti-inflammatory effect, improves immunity. Prednisolone is administered intravenously (drip).
Treatment with corticosteroids helps to stop inflammatory process, however, does not eliminate the cause of the disease. For normal mental and physical development newborns are prescribed mineralocorticoids. It is recommended to add to food table salt. Nutrition during treatment should be complete and balanced, rich in vitamins.
If drug treatment does not have the desired effect, you should resort to surgical intervention.
How to prevent disease
Prevention of the adrenal glands in a child great importance, because it allows you to prevent the disease and avoid serious consequences. First of all, it is necessary to minimize the occurrence stressful situations, the occurrence of which adversely affect the nervous system. General hardening child, good nutrition baby and taking vitamins will minimize the occurrence of the disease. Spend more time on your health and the health of your children. Be healthy and happy!
Primary adrenal insufficiency may have genetic causes and, although not necessarily, manifests itself in infancy. It can also be acquired, for example, with an autoimmune lesion of the adrenal cortex. However, a predisposition to autoimmune pathology often also has a genetic nature, and therefore such a distinction is very relative.
Congenital adrenal insufficiency.
Congenital defects in steroidogenesis. The most common cause of adrenal insufficiency in infancy is congenital hyperplasia of the adrenal cortex with salt loss. Approximately 75% of children with 21-hydroxylase deficiency, almost all children with lipoid hyperplasia of the adrenal cortex, and most children with 3R-hydroxysteroid dehydrogenase deficiency show symptoms of salt loss after birth, since in these cases the synthesis of not only cortisol, but also aldosterone is impaired.
Congenital adrenal hypoplasia.
Adrenal insufficiency in such cases is usually manifested acute symptoms immediately after birth, but sometimes they occur only in late childhood or even in adulthood. At histological examination hypoplastic adrenal cortex reveals its disorganization and cytomegaly. This pathology occurs mainly in boys and is caused by a mutation of the DAX1 gene, located on the Xp21 chromosome and involved in the synthesis of nuclear hormone receptors. In boys with such a defect, due to secondary hypogonadism, there is no sexual development. The basis of secondary hypogonadism is a mutation of the same DAX1 gene. The cryptorchidism often seen in these boys is probably early sign secondary hypogonadism.
Congenital adrenal hypoplasia can also be a manifestation of gene sequence syndrome, combined with muscular dystrophy Duchenne, glycerokinase deficiency and/or mental retardation.
Adrenoleukodystrophy.
With this pathology of adrenal insufficiency, demyelination of CNS neurons is accompanied. Due to disruption of peroxisomal oxidation in tissues and biological fluids accumulate fatty acid with very long chain.
The most common is the X-linked form of adrenoleukodystrophy (X-ALD) with varying penetrance. The disease is most often manifested by degenerative neurological disorders in childhood or adolescence, progressing to severe dementia with impaired vision, hearing, speech and gait. After a few years, death occurs. A milder form of X-ALD, adrenomyeloneuropathy, begins in late adolescence or early adulthood. Adrenal insufficiency often develops long before onset neurological symptoms and may be the only manifestation of the disease. This disease is based on mutations in the ABCD1 gene located on the Xq28 chromosome. The gene encodes a transmembrane transporter involved in the transfer of very long chain fatty acids into peroxisomes.
More than 400 mutations of this gene have been found in patients with X-ALD. Members of the same family usually have the same mutation, although the course of the disease may vary. This is probably due to the action of modifier genes or some other factors. The degree of neurological disorders and the severity of adrenal insufficiency do not correspond to each other. Currently, prenatal diagnosis of this disease is possible using DNA testing or determination of the level of very long chain fatty acids in relatives. In women - heterozygous carriers of the defective gene, symptoms of the disease occur relatively late. Adrenal insufficiency develops rarely. For treatment use:
glycerol trioleate or glycerol trierucate (Lorenzo's oil);
bone marrow transplant;
lovastatin;
fenofibrate;
gene therapy.
However, the effectiveness of all these tools and methods is still unknown.
Neonatal ALD is a rare autosomal recessive disorder. It is characterized by neurological disorders and dysfunction of the adrenal cortex. Most patients have severe mental retardation and they die before 5 years of age. This disease is a type of Zellweger's syndrome (cerebrohepatorenal syndrome), in which, due to mutations of certain genes that control the formation of peroxisomes, the latter are completely absent.
Familial glucocorticoid deficiency.
This form of chronic adrenal insufficiency is characterized by an isolated deficiency of glucocorticoids, increased level ACTH and normal secretion aldosterone. The salt loss seen in most other forms of adrenal insufficiency is absent here. At an early age, patients develop hypoglycemia and convulsions, there is increased skin pigmentation. This autosomal recessive disorder occurs with equal frequency in boys and girls. The adrenal cortex is atrophied, but its glomerular zone is relatively preserved. Some (about 40%), but not all patients have various mutations in the gene encoding the ACTH receptor.
Resistance to ACTH is also observed in Allgrove's syndrome (three A syndrome), in which such resistance is combined with achalasia of the cardia of the stomach and alacrymia. Autonomic dysfunction, mental retardation, deafness, and motor neuropathy often occur with this disease. Allgrove's syndrome is also inherited in an autosomal recessive manner; the gene is mapped to chromosome 12ql3.
Disorders of the synthesis and metabolism of cholesterol.
The diseases of this group include abetalipoproteinemia with deficiency of apoB-containing lipoproteins, and familial hypercholesterolemia with impaired LDL receptors. In patients with this pathology, a decrease in the function of the adrenal cortex was found. Adrenal insufficiency has been found in Smith-Lemli-Opitz syndrome, an autosomal recessive disorder with facial abnormalities, microcephaly, limb abnormalities, and developmental delay. In this syndrome, mutations of the Ilql2-ql3 gene located on the chromosome, which encodes D7-sterol reductase, were detected. As a result, the final stage of cholesterol synthesis is disturbed and its level decreases, the content of 7-dehydrocholesterol sharply increases and adrenal insufficiency develops. Wolman's disease is a rare autosomal recessive pathology, which is based on mutations in the gene encoding lysosome acid lipase. This leads to the accumulation of cholesterol esters in the lysosomes of most organs and, ultimately, to a violation of their function. In children on the 1st-2nd month of life, hepatosplenomegaly, steatorrhea, bloating and developmental delay are noted. Revealed adrenal insufficiency and bilateral calcification of the adrenal glands. Patients usually die in the first year of life. The gene encoding lysosome acid lipase is located on chromosome 10q23.2-23.3 and is known to mutate in Wolman's disease.
Deficiency of transcortin and a decrease in its affinity for cortisol.
In this pathology, plasma cortisol levels are reduced, but urinary free cortisol and plasma ACTH levels are normal. Adults with transcortin deficiency often have hypotension and fatigue.
Acquired adrenal insufficiency
Autoimmune Addison's disease.
Acquired primary adrenal insufficiency is most often associated with autoimmune destruction of the glands. The adrenal glands are sometimes so reduced in size that they are not visible at autopsy, and only remnants of their tissue can be found on microscopic sections. The medulla is usually preserved, and lymphocytes are abundant in place of the cortex. In advanced cases, the secretion of all hormones of the cortical substance drops out, but in the early stages only cortisol deficiency is possible. In most patients, plasma antibodies to cytoplasmic antigens of cells of the cortical substance are present, the autoantigen is most often 21-hydroxylase (CYP21).
Addison's disease is often a component of two autoimmune polyglandular syndromes. The first manifestation of autoimmune polyglandular syndrome type I is usually chronic candidiasis of the skin and mucous membranes. Then hypoparathyroidism develops and, finally, primary adrenal insufficiency, which in typical cases manifests itself in early adolescence. To other commonly associated autoimmune disorders include gonadal insufficiency, alopecia, vitiligo, keratopathy, tooth enamel hypoplasia, nail dystrophy, intestinal malabsorption, and chronic active hepatitis. Hypothyroidism and type I diabetes occur in less than 10% of patients. Some components of this syndrome can appear even after 40 years. The presence in patients of antibodies to the tissue of the adrenal glands and to steroid-producing cells indicates high probability development of Addison's disease or (in women) ovarian failure. Adrenal insufficiency in autoimmune polyglandular syndrome type I can develop quite quickly. Cases of unexpected death of patients and their brothers or sisters are described, which emphasizes the need for careful monitoring of patients and a detailed examination of even their apparently healthy close relatives.
Among autoantibodies to steroidogenesis enzymes, antibodies to CYP21, CYP17 HCYPHAI are found. The syndrome is inherited in an autosomal recessive manner, and the defect is found in a gene located on chromosome 21q22.3, called autoimmune regulator-1 (AIRE1). This gene encodes a transcription factor that plays an important role in the regulation of the immune response. Approximately 40 different mutations of the AIRE1 gene have been identified in patients with autoimmune polyglandular syndrome type I, with two of them (R257X and divisions of three base pairs) occurring most frequently. In one family with the G228W missense mutation, the disease was inherited in an autosomal dominant manner.
Autoimmune polyglandular syndrome type II is a combination of chronic adrenal insufficiency with an autoimmune lesion. thyroid gland(Schmidt's syndrome) or type I diabetes mellitus (Carpenter's syndrome). These disorders are sometimes accompanied by gonadal insufficiency, vitiligo, alopecia, and chronic atrophic gastritis with or without Addison-Birmer disease. Among these patients, HLA-D3 and HLA-D4 (risk markers) occur with an increased frequency. The MHC class I genes MICA and MICB are also associated with autoimmune polyglandular syndrome type II. This syndrome is typical mainly for middle-aged women and can be passed from generation to generation. Patients also have antibodies to the adrenal antigens CYP21, CYP17, and CYP11A1.
Infections.
In the past, destruction of the adrenal glands was very often associated with their tuberculous lesion, but at present this is much less common. The most common infectious cause adrenal insufficiency - meningococcemia. Hypoadrenal crisis that develops with fulminant meningococcal sepsis is called the Waterhouse-Frideriksen syndrome. Patients with AIDS may have various violations HHNS, but overt adrenal insufficiency rarely develops. However medicines used in AIDS may affect adrenal function.
Medicines.
The antifungal agent ketoconazole causes adrenal insufficiency by inhibiting the activity of steroidogenesis enzymes. Rifampicin and anticonvulsants (phenotoin and phenobarbital) reduce the effectiveness of corticosteroid replacement therapy by inducing the synthesis of steroid metabolism enzymes in the liver. Mitotane, used in adrenal cancer and Cushing's syndrome, has toxic effect on the adrenal cortex, and also affects the peripheral metabolism of cortisol. Signs of adrenal insufficiency appear in a significant number of patients receiving mitotane.
Hemorrhage in the adrenal glands.
In newborns, this may be due to difficult births (especially with the breech presentation of the fetus), but it is not always possible to establish the cause. Such cases occur with a frequency of 3:100,000 live births. Sometimes the hemorrhage is so significant that it leads to death from blood loss or acute adrenal insufficiency. With a hemorrhage in the adrenal glands, a mass in the abdomen is palpated, unexplained jaundice or hematoma of the scrotum develops. Often, symptoms appear only after calcification of the adrenal glands. Cases of hemorrhage in the adrenal glands have been described in prenatal period. The most common cause of hemorrhage in postnatal life is the use of anticoagulants. In children, this may be due to accidental poisoning with such agents.
A syndrome such as adrenal insufficiency in children is quite rare, does not have characteristic manifestations and poses a serious threat to the life of the child. The disease can be either congenital or acquired. Hypofunction of the adrenal glands is manifested in the fact that the hormones that are secreted by the organs are produced in not enough. This entails failures in metabolic processes up to the development of deadly processes.
The structure and function of the adrenal glands in children
The organs are composed of two layers: the cortical outer layer and the medulla inner. The functional part of the organ cortex is responsible for the processing of steroids. The interaction of steroids and cholesterol ensures the production of hormones, the main function of which is the regulation of metabolism in the body. Thus, the adrenal glands are involved in the production of complex biological substances, the analogues of which do not exist in nature.
Every child from the moment of birth to the tenth day of life, there is a threat of the development of pathology as adrenal insufficiency.
Possible manifestations of the disease
congenital agenesis, fusion
Congenital aplasia of the kidneys in young children is rare disease. Deviation is the lack of development of the organ. It is diagnosed quite rarely, but in cases of confirmation of the diagnosis, the outcome of the disease is fatal. More in more rare cases there is a weak development of one organ, but if the second organ completely takes over the work of the first, then the manifestation of the problem may be absent altogether. Pathologies such as fusion of the adrenal glands have been recorded. Fusion can occur both with the aorta (in the anterior or posterior part) and with nearby authorities- liver or kidneys. In case of abnormal development of the abdominal wall, the adrenal glands can fuse even with the lungs.
Adrenoleukodystrophy
Refers to a hereditary pathology, which manifests itself in the destruction nerve cells brain at the level of the membrane. With adrenoleukodystrophy, the body cannot break down fatty acids on its own, which leads to damage to the nervous system and malfunction of the adrenal glands. According to statistics, such a pathology affects more often men, but women are the carrier that predisposes to the development of pathology. In children, the pathology develops rapidly, and if treatment is not started on time, death occurs in 7-10 years.
Adrenal insufficiency
Acute and chronic pathology is dangerous sharp decline necessary hormonal substances. As a result of this process, the concentration of chloride and sodium ions decreases, due to the impossibility of their absorption into the intestine. Gradually, there is a decrease in fluid in the body, blood thickens, and the function of filtering from toxic substances weakens in the kidney. When such disturbances in the production of hormones are of a permanent nature, the pathology becomes chronic.
Primary hyperaldosteronism
Excess aldosterone provokes the appearance of primary hyperaldosteronism. The main problem with this process is the excess production of aldosterone, which is responsible for the excretion of potassium by the kidneys and the regulation of sodium levels. When the hormone is produced in excess, the action of the enzyme renin is disrupted, which, in turn, is responsible for vasoconstriction and balancing blood pressure. As a result, the pressure rises sharply, the process of deficiency develops. useful elements(magnesium and potassium). Insufficient concentration of potassium in the blood leads to cardiac pathologies, headaches and night cramps. Against the background of this process, calcium levels decrease, which is dangerous with bouts of lack of air, numbness of the arms and legs.
Preparations with high content potassium and calcium in this case are ineffective, since the concentration of elements in the body is sufficient, but due to the pathological activity of the adrenal glands, they are not absorbed.
Congenital adrenal hyperplasia
This pathology entails a whole range of various problems. There are malfunctions in the work of the adrenal cortex and, as a result, the protein is blocked. As a result of this process, the patient experiences hyperplasia, which consists in an increase in the number of cells, after which the organ increases in size. The disease leads to the development of adrenal insufficiency, which provokes a violation in the development of children in the future, up to the appearance of infertility.
Hemorrhage
Hemorrhage is a common problem. In case of infection, sepsis, or various kinds of birth injuries, premature babies may experience bleeding in the adrenal glands. Similar problems lead to very serious consequences: from death individual sections tissue before cyst formation. There are cases of bilateral hemorrhages, such moments usually cause the death of the patient.
Tumor formations
If a we are talking about tumor lesions of the adrenal glands, then, as a rule, this benign formations. Such neoplasms tend to grow in both the outer and inner layers. The main method of treating pathological formations is their surgical removal. In this case, the patient also cannot do without supporting drug therapy. If you start the process, the tumors will grow, and this can lead to the development diabetes, kidney failure and other pathologies that are life-threatening for the patient.
Cancer (adrenocortical)
Oncological tumor - occurs in children adolescence. Adrenocortical cancer is difficult to diagnose and yet harder to treat. Children under 6 years of age are at risk, in very rare cases, pathology is recorded in adolescents under 15 years of age. Say what exactly provokes the disease, and what characteristic symptoms manifestations are difficult, so it is most often diagnosed on late term when the treatment loses its effectiveness.
Pheochromocytoma
Pathologies provoked by such a tumor are quite rare. Because of the disease in the child's body, catecholamine substances begin to be synthesized excessively. All higher nervous processes, as well as physical activity regulated by metabolic processes. Any failures associated with catecholamines can lead to serious pathologies nervous, endocrine and cardiovascular systems.
The endocrine system is the main conductor of all processes in the body: metabolism, maintenance vital functions, growth and development. Excess or lack of functioning of the endocrine glands will inevitably lead to serious problems. Hormonal imbalances involve pathological process all organs without exception. To similar diseases include adrenal insufficiency.
Concept definition
The endocrine system is a multifaceted mediator of the brain in the management of organs and tissues. The orders of the nervous system of the endocrine glands translate into their own language special chemical compounds- hormones.
central authorities endocrine system is the hypothalamus. In its special cells, substances of a protein nature are produced - releasing factors. The latter are sent to carry out the first line of management of organs at the level of the pituitary gland. This endocrine gland is directly located in the cranial cavity near the brain.
The pituitary gland, in turn, acts as the second line of regulation of the body's activities. The latter is carried out with the help of chemical compounds - tropic hormones. These substances directly affect the third level of the endocrine system - the endocrine glands.
Adrenal hormone secretion is regulated by adrenocorticotropic hormone
Among the glands of the endocrine system, the adrenal gland stands out for its special role. This organ is paired, located in abdominal cavity. AT anatomical structure adrenal cortex and medulla are secreted. The first is further divided into zones.
The glomerular layer is responsible for the production of mineralocorticoids responsible for the balance of sodium and potassium in the body. The bundle zone of the cortex produces glucocorticoids that control carbohydrate metabolism, the level blood pressure, formation of adipose tissue, activity immune system. The reticular layer is the source of both male and female sex hormones in people of both sexes. The medulla is responsible for the production of anxiety and stress hormones - adrenaline and norepinephrine.
Adrenal hormones regulate all components of metabolism
Direct control over the activity of the adrenal glands is carried out by the pituitary gland through the release of adrenocorticotropic hormone (ACTH).
Adrenal insufficiency is a medical term that describes the unreasonably low production of all hormones by this endocrine gland, leading to a significant metabolic disorder in the body.
Synonyms of the disease: hypocorticism, Addison's disease, bronze disease, adrenal hypofunction.
There is Addison-Birmer disease, which has nothing to do with adrenal insufficiency. With this pathology, perverted hematopoiesis is observed in bone marrow leading to anemia, stomach damage and nervous tissue due to a lack of cyanocobalamin (vitamin B12).
Classification of adrenal insufficiency
Hypofunction of the adrenal glands is divided into several varieties.
- According to the nature of the course, hypocorticism is divided into:
- acute form, in which pronounced changes in metabolism are formed in a short period of time;
- Addisonian crisis is a fulminant form of the disease in which a high rate of metabolic disturbance can lead to a life-threatening condition;
- chronic form characterized by slow development clinical signs;
- According to the level of occurrence hormonal disorders hypocorticism is subdivided into:
- primary, at which the level hormonal disorders limited to the adrenal gland itself;
- secondary, characterized by a disorder in the release of ACTH by the pituitary gland;
- tertiary, in which hormonal background changes at the level of releasing factors of the hypothalamus;
- iatrogenic, occurring after surgical removal glands;
- transient, characterized by inconstancy of hormonal disorders.
Secondary and tertiary adrenal insufficiency are logically combined by the term central hypocorticism, emphasizing high level localization of the pathological process.
Causes and development factors
The causes of primary, secondary and tertiary forms of adrenal insufficiency are somewhat different.
Causes of hypocorticism depending on the level of damage to the endocrine system - table
| Primary hypofunction | Secondary hypofunction | Tertiary hypofunction |
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Causes of hypocorticism - gallery
Tumor lesion of the pituitary gland - the cause of hypocorticism 
Hemorrhage in the adrenal glands - serious complication infectious diseases
Cerebral ischemia or hemorrhage can lead to the development of central hypocorticism
Any process leading to adrenal insufficiency will inevitably lead to a multifaceted metabolic disorder. Decreased production of mineralocorticoids leads to massive excretion of sodium by the kidneys and potassium retention. The balance of these substances is extremely important for the proper functioning of the body: heart rhythm, gastrointestinal tract, nervous tissue of the brain.
A low level of glucocorticoids will inevitably lead to a decrease in the amount of glucose in the blood (hypoglycemia), muscle weakness, low level blood pressure (hypotension) and discoloration of the skin.
Hypoglycemia is a frequent companion of adrenal insufficiency.
The lack of the level of sex hormones produced by the reticular zone in women stops the growth of pubic hair and in armpit. In men similar violation do not cause a vivid clinical picture, because the right substances synthesize male glands internal secretion (testes).
A special group is made up of pregnant women with chronic adrenal insufficiency. Hypocorticism affects negatively both the course of pregnancy and the process of childbirth, and the health of the newborn. During pregnancy, women are at increased risk of developing acute form diseases, as a result of which the dose of hormones necessarily increases. Offensive labor activity requires active medical support with drugs. Newborns often suffer from lack of weight, anatomical developmental anomalies. Stillbirth is not ruled out.
Clinical picture
Symptoms of various forms of adrenal insufficiency are somewhat different.
Acute hypocorticism usually develops within a few hours and is characterized by the following symptoms:
Decompensation of chronic adrenal insufficiency leads to the appearance of similar symptoms. Without immediate medical care this clinical picture progresses rapidly and leads to death.
Chronic adrenal insufficiency in the subcompensated stage is characterized by a slow course and smoothing of the following clinical signs:
A compensated form of hypocorticism is observed against the background of adequate therapy. Clinical picture disease is characterized by normalization of body weight, muscle tone, stool and blood pressure.
The following factors most often contribute to the decompensation of the disease:
In newborns, hypocorticism is part of a hereditary pathology - adrenogenital syndrome. The disease in this case manifests itself by the development of an Addisonian crisis. All babies are screened for the condition immediately after birth (neonatal screening).
Clinical aspects of Addison's disease - video
Diagnosis and differential diagnosis
To establish the correct diagnosis, the following measures are necessary:
Differential diagnosis of adrenal insufficiency is carried out with the following diseases:
Methods of treatment of adrenal insufficiency
Treatment of hypocorticism is carried out under the guidance of an endocrinologist and is complex.
Help with acute adrenal insufficiency
The acute form of the disease (Addisonian crisis) needs the timely elimination of pathological metabolism in the intensive care unit through the following measures:
- intravenous administration of sufficient doses of glucocorticoids;
- maintenance of vital functions: respiratory and cardiac activity;
- correction of electrolyte metabolism by intravenous administration sodium chloride solution;
- correction carbohydrate metabolism by injecting glucose solutions.
Drug treatment of the chronic form
At chronic form hypocorticism, the basis of treatment is substitution therapy. Hormonal preparations, as a rule, have a combination of glucocorticoid and mineralocorticoid effects in varying degrees. Often, patients are prescribed a combination of several hormonal drugs from the list below:
For treatment, as a rule, tablet forms of drugs are used. Most of daily dose taken in the morning and only a small amount - at lunchtime. This method allows you to save the natural fluctuations of the hormonal background.
Diet
- increase in total calories;
- increase in protein content;
- fractional consumption of carbohydrate foods;
- additional intake of salt;
- restriction of foods containing a large amount of potassium: potatoes, raisins, tangerines, prunes;
- sufficient content of vitamins;
- consumption of fresh fruits.
Physiotherapeutic methods are used to correct the disease that led to the development of adrenal insufficiency. Specific methods are selected by the doctor depending on the underlying diagnosis.
Traditional medicine and homeopathy in the treatment of hypocorticism have not proven their effectiveness, therefore, they are not used in therapy.
Complications and prognosis
The prognosis for the treatment of adrenal insufficiency largely depends on the form of the disease and the adequacy of therapy. Life expectancy with hypocorticism of tuberculous nature and timely treatment reaches twenty years of age. Adrenal gland disease, caused by problems in the functioning of the immune system, proceeds unfavorably. In all other cases, a properly selected dose of hormones ensures a normal life expectancy. At severe course The disease may develop the following complications:
- collapse;
- heart rhythm disturbances;
- cerebral edema;
- fatal outcome;
- overdose of hormonal drugs;
Patients with chronic adrenal insufficiency, depending on the severity of the course, the severity of the clinical signs of the disease and the success of hormonal treatment, can be assigned the first, second or third disability groups.
Prevention
Prevention of adrenal insufficiency includes the following activities:
- neonatal screening;
- timely treatment of the tuberculosis process, injuries and infectious diseases;
- vaccination against infectious diseases.
Insufficiency of the adrenal cortex - serious illness leading to death if left untreated. Compliance with all medical advice- pledge successful therapy this disease.
