What is primary, secondary and tertiary hyperparathyroidism. Secondary hyperparathyroidism: causes, symptoms and treatments
Hyperparathyroidism– a chronic endocrine disease caused by increased production of the hormone steam thyroid glands(parathyroid hormone, parathyroidocrine, PTH) for tumors (adenomas) of the parathyroid glands, or their hyperplasia (non-tumor growth of organ tissue, accompanied by an increase in working function).Anatomy of the parathyroid glands
The typical number of parathyroid glands is two pairs (observed in 80-85% of people). 15-20% of the world's population have from 3 to 12 glands. Congenital variations in the number of glands are usually not accompanied by pathological symptoms.With a typical number of parathyroid glands, the weight of one gland normally ranges from 20 to 70 mg, and the dimensions, as a rule, do not exceed 6 * 3 * 1.5 mm. The glands are disc-shaped and differ from the thyroid tissue by their yellow color.
The parathyroid glands are most often located on the posterior surface of the thyroid gland. Moreover, their upper pair is located at the level of the upper and middle third of the thyroid gland, and the lower pair is at the level of its lower third.
However, the location of the parathyroid glands is highly variable. For example, the lower pair may be located in the mediastinum, maintaining connection with thymus gland(thymus), sometimes inside the gland itself.
Usually, parathyroid glands located at the entrance to the thyroid gland blood vessels or nerve branches. Often they are partially or completely immersed in the thyroid tissue, although they have their own capsule.
Additional parathyroid glands may be located in the anterior and posterior mediastinum, inside the tissue of the thyroid and thymus glands, on the posterior surface of the esophagus, in the pericardial cavity (in the heart sac), in the area of the bifurcation of the carotid artery.
The parathyroid glands are supplied with blood from the branches of the arteries of the thyroid gland, so they can be damaged during operations on the thyroid gland.
Functions of parathyroid hormone
Despite their small size, the parathyroid glands play important role in the life of the body, regulating the exchange of calcium and phosphorus. Parathyroid hormone promotes the release of calcium from bone tissue and thus increases its concentration in the blood plasma. In addition, parathyroid hormone increases calcium absorption in the intestine and reduces its excretion in the urine, which also leads to hypercalcemia.
An increase in the production of parathyroid hormone contributes to an increase in the excretion of phosphorus from the body in the urine (phosphaturia).
Regulation of the activity of the parathyroid glands occurs according to the principle feedback: with a decrease in the level of calcium in the blood, their activity increases, with hypercalcemia it decreases. Thus, with excess calcium in the blood, the size of the glands decreases, and prolonged hypocalcemia leads to working hyperplasia of the organs - an increase in their activity and size.
Pathological physiology
Increased secretion of parathyroid hormone leads to the leaching of calcium from the bones and an increase in its concentration in the blood plasma (hypercalcemia).Pathology skeletal system in hyperparathyroidism, it manifests itself in systemic skeletal fibrosis (replacement of bone tissue with fibrous tissue), accompanied by gross deformations of the skeletal system.
Due to the high level of calcium in the blood, calcifications form in the internal organs (in medicine this phenomenon is called “transfer of the skeleton to soft tissue”). In this case, the kidneys and vessel walls suffer the most. So in severe cases, patients die from kidney failure or severe circulatory disorders.
Another common complication is the formation of calcium phosphate stones in upper sections urinary system, which further aggravates the kidney condition.
Calcium plays an important role in water-electrolyte metabolism, therefore an increase in its concentration causes systemic effects, such as:
- conduction disturbance in nerve tissue, which ultimately leads to muscle weakness, depression, memory and cognitive impairment;
- arterial hypertension ;
- increased gastric secretion, which can be complicated by the formation of stomach ulcers and duodenum.
Epidemiology
Among endocrine diseases hyperparathyroidism ranks third in prevalence (after diabetes mellitus and hyperthyroidism).Women get sick 2-3 times more often than men. With age, the likelihood of developing the disease increases (especially after menopause in women). As life expectancy increases in highly developed countries, the incidence of hyperparathyroidism is likely to increase.
Causes
According to the cause of increased levels of parathyroid hormone in the blood, there are:1. Primary hyperparathyroidism.
2. Secondary hyperparathyroidism.
3. Tertiary hyperparathyroidism.
4. Pseudohyperparathyroidism.
The clinical symptoms of all forms of hyperparathyroidism are largely similar, since in all the cases described above, calcium is washed out from the skeletal system, and persistent hypercalcemia develops, leading to many complications.
However, the clinical picture will also be influenced by the underlying disease that caused the hyperparathyroidism syndrome. Treatment tactics in many cases will be different.
Primary hyperparathyroidism
Primary hyperparathyroidism is a syndrome of increased secretion of parathyroid hormone caused by the primary pathology of this endocrine organ. In 85% of cases primary hyperparathyroidism The cause of the development of the pathology is a single benign tumor (adenoma) of one of the glands. Less common (in 5% of cases) are multiple adenomas affecting several glands. Even less common is parathyroid cancer.
Cases of primary diffuse hyperplasia of the parathyroid glands are extremely rare (we are often talking about hyperplasia of accessory glands located in the mediastinum, which significantly complicates topical diagnosis).
Typical adenomas develop predominantly in older people, most often in women during menopause. Rare cases diffuse hyperplasia is characteristic of young and, as a rule, are combined with other diseases endocrine system. Parathyroid cancer often develops after radiation to the head and neck.
Secondary hyperparathyroidism
Secondary hyperparathyroidism is a syndrome of increased concentrations of parathyroid hormone in the blood, which develops in initially healthy parathyroid glands due to reduced calcium levels caused by other diseases.Thus, in response to a lack of calcium in the blood, parathyroid hormones are released, causing hypercalcemia - this normal regulation based on the feedback principle. However, in cases where we are talking about severe chronic diseases leading to a sharp and prolonged decrease in the level of calcium in the blood, it may develop over time serious pathology– secondary hyperparathyroidism.
The most common causes of secondary hyperparathyroidism are severe kidney pathology and malabsorption syndrome (impaired absorption of nutrients in the gastrointestinal tract) - renal and intestinal (intestinal) forms of secondary hyperparathyroidism are respectively distinguished.
In patients undergoing hemodialysis (device " artificial kidney"), secondary hyperparathyroidism syndrome develops in 50-70% of cases. In patients who have undergone gastrectomy, the level of parathyroid hormone increases in 30% of cases.
The development of secondary hyperparathyroidism in chronic renal failure is associated with impaired synthesis of active vitamin D in the renal parenchyma, which leads to impaired calcium absorption and hypocalcemia.
Hypocalcemia in intestinal forms of secondary hyperparathyroidism is associated with impaired absorption of vitamin D and calcium in the gastrointestinal tract.
In addition, hypocalcemia develops when serious illnesses liver (primary biliary cirrhosis) due to impaired vitamin D metabolism, as well as rickets and rickets-like diseases in children.
Tertiary hyperparathyroidism
Tertiary hyperparathyroidism was first described in patients who had successfully undergone kidney transplantation. It turned out that complete restoration of all renal parameters did not eliminate increased level parathyroid hormone in the blood and, accordingly, the symptoms of secondary hyperparathyroidism.Thus, with prolonged stimulation of the parathyroid glands by a state of hypocalcemia, irreversible working hyperplasia of their glandular tissue develops. This condition was called tertiary hyperparathyroidism.
The clinic of tertiary hyperparathyroidism is in many ways similar to the clinic of primary parathyroid hyperplasia.
Pseudohyperparathyroidism
Pseudohyperparathyroidism is a hypercalcemia syndrome associated with the development malignant tumors(some malignant neoplasms lungs, kidneys, mammary glands, multiple myeloma). Often, pseudohyperparathyroidism is caused by the presence of parathyroid hormone-producing cells in the tumor. However, according to the latest scientific data, this is not the main and not the only cause of this pathology.The fact is that an increase in the level of calcium in the blood is often not combined with a high level of parathyroid hormone. It is believed that some tumors produce substances similar in action to parathyroid hormone. Hypercalcemia is often associated with local (cancer metastases) or diffuse (myeloma) effects of the tumor on bone tissue, accompanied by bone dissolution and the release of calcium into the blood.
Symptoms
Symptoms of primary hyperparathyroidism
Initial manifestations of the disease
Often the initial stages of primary hyperparathyroidism are asymptomatic, which causes late diagnosis pathology. In such cases, the level of parathyroid hormone and calcium in the blood is usually low, and are almost the only signs of the disease. Therefore, in most cases, pathology is determined by chance, during examination for other diseases. Up to 80% of patients in initial stage diseases present nonspecific complaints, such as:
- weakness;
- lethargy;
- tendency to constipation;
- Bad mood;
- pain in bones and joints.
In more severe cases, the first manifestations of the disease are usually complaints from muscular system. A high concentration of calcium in the blood disrupts neuromuscular transmission and leads to a kind of myopathy.
First, weakness and pain appear in certain muscle groups, most often in the lower extremities. Patients complain of difficulties with normal walking (often tripping, falling), it is difficult for them to get up from a chair (support is required), get on a tram, bus, etc.
Looseness in the joints appears early, a duck's gait and pain in the feet are very characteristic (flat feet develop due to impaired tone of the foot muscles). Thus, with severe primary hyperparathyroidism, patients find themselves bedridden even before pathological fractures appear.
Often the onset of the disease is the appearance of a peculiar diabetes mellitus(polyuria and polydipsia with reduced urine density). This syndrome associated with damage to the kidney tubules by massive calcium release (calciuria) and loss of their sensitivity to antidiuretic hormone.
A characteristic early manifestation of damage to the skeletal system in primary hyperparathyroidism is the loosening and loss of healthy teeth.
In severe cases of the disease, severe weight loss develops, associated with a sharp loss of appetite and polyuria, leading to dehydration. Sometimes patients lose up to 10-15 kg in the first 3-6 months of the disease. Characterized by sallow color and dry skin. Such appearance is associated both with general exhaustion and dehydration, and with the development of anemia, the genesis of which in primary hyperparathyroidism is still not fully understood.
Advanced clinical stage of the disease
Symptoms of the advanced clinical stage of primary hyperparathyroidism in severe and moderate cases can be divided into the following groups:
- bone;
- articular;
- renal;
- gastrointestinal;
- vascular;
- ophthalmological;
- neurological.
Skeletal lesion
Under the influence of parathyroid hormone, calcium and phosphorus are intensively washed out of the bones, and osteoclasts, bone tissue cells that promote bone dissolution, are also activated. As a result, osteoporosis occurs (partial resorption of bone tissue). Osteoporosis can be either diffuse (a general decrease in bone density) or limited (subperiosteal osteoporosis - a decrease in bone density directly under the periosteum). It can also manifest itself through the formation of cysts (cavities in the bones that can be filled with fluid).
Osteoporosis causes severe bone deformation. Thus, the pelvis takes the shape of a “card heart”, the femur – a “shepherd’s stick”, the chest becomes like a bell. As a rule, the spine suffers, especially the thoracic and lumbar vertebrae, which take on a “fish” shape. General deformation occurs due to pathological changes in the vertebrae spinal column– kyphosis (posterior curvature with the formation of a hump), scoliosis (sideways curvature with a violation of the overall symmetry of the body), kyphoscoliosis.
Due to a decrease in bone density, bone fractures occur even with a slight load, or spontaneously (pathological fractures). Unlike ordinary fractures, pathological fractures are less painful, which in some cases complicates diagnosis and leads to improper fusion of bones, or the formation false joints(bone fragments do not heal, but acquire pathological mobility), which leads to permanent disability. Healing of fractures is slow. However, if the bones fuse, a fairly dense callus, so there are no repeated fractures in the same place.
Due to severe bone deformities, patients may lose 10-15 cm or more in height.
Joint damage is associated with both skeletal deformation and the deposition of calcium phosphate salts (false gout). In addition, increased levels of parathyroid hormone can stimulate the deposition of uric acid crystals in the joints (true gout).
Defeat nervous system, blood vessels and internal organs
The second target after bone in primary hyperparathyroidism is the kidneys. Expressiveness kidney symptoms, as a rule, determines the prognosis of the disease, since renal failure that develops as a result of prolonged hypercalcemia is irreversible.
Direct damage to the renal parenchyma is aggravated by the formation of calcium phosphate stones in the upper urinary system. Characteristic are coral stones that fill the entire pyelocalyceal system kidneys
Lesions of the gastrointestinal tract are manifested by symptoms such as:
- nausea;
- a sharp decline appetite;
- tendency to constipation.
Further development ulcerative lesions: duodenal ulcers are most common; the stomach, esophagus and intestines are less commonly affected. In severe cases, erosive processes occur in many organs of the gastrointestinal tract with the formation of multiple ulcers. Characterized by a tendency to bleeding, frequent exacerbations and relapses.
Calculous cholecystitis (formation of stones in gallbladder), calcification of the pancreatic ducts with the development of pancreatitis. It is typical that with the development of pancreatitis, the level of calcium in the blood decreases due to increased production of the hormone glucagon by the inflamed gland.
Pathology of cardio-vascular system in primary hyperparathyroidism is associated with arterial hypertension and calcium deposition in the walls of blood vessels. In severe cases, damage to many organs may develop due to disruption of their blood supply.
Eye damage is caused by the deposition of calcium salts in the cornea (ribbon keratopathy).
Pathology of the nervous system manifests itself already in the early stages of the disease, and the severity of symptoms strongly depends on the level of calcium in the blood. Characteristic:
- depressive states varying degrees expressiveness;
- apathy;
- decreased memory and cognitive abilities;
- in severe cases – confusion, psychosis.
Symptoms of secondary and tertiary hyperparathyroidism
Secondary hyperparathyroidism occurs against the background of a disease that causes an increase in the production of parathyroid hormone (most often this is a kidney pathology).Since the cause of secondary hyperparathyroidism was a pathology that provoked prolonged hypocalcemia, and overproduction of parathyroid hormone was a kind of compensatory reaction, the level of calcium in the blood in such patients is usually within normal limits.
Most characteristic symptoms secondary hyperparathyroidism are lesions on the skeletal system, since secondary hypothyroidism develops against the background of a lack of vitamin D, accompanied by the leaching of calcium from the bones, and parathyroid hormone enhances this process.
Among extraosseous manifestations, the most common are calcifications in soft tissues and the walls of large vessels. Eye damage in the form of calcification of the conjunctiva and cornea, combined with chronically recurrent conjunctivitis, is very typical.
Tertiary hyperparathyroidism develops with a long course of secondary hyperparathyroidism, so that even with the elimination of the disease that caused hypocalcemia and stable normalization of calcium levels in the blood, the production of parathyroid hormone remains elevated due to irreversible working hyperplasia of the parathyroid glands.
Symptoms of tertiary hyperparathyroidism are nonspecific and are in many ways similar to secondary hyperparathyroidism. High levels of parathyroid hormone and calcium in the blood are determined in the laboratory.
Hypercalcemic crisis
Hypercalcemic crisis is a rather rare complication of hyperparathyroidism, caused by a sharp increase in calcium levels in the blood, and characterized by severe disturbances of higher nervous activity, a deadly increase in blood clotting, up to the development of thrombosis and disseminated intravascular coagulation syndrome (DIC), as well as the development of acute cardiovascular failure, which can cause cardiac arrest. If hyperparathyroidism is complicated by a hypercalcemic crisis, the mortality rate of patients can reach 50-60% or higher.
Most often, the factors provoking the development dangerous complication, are:
- exacerbation of primary hyperparathyroidism in the absence of adequate treatment;
- spontaneous pathological bone fractures (massive release of calcium into the blood);
- dehydration;
- immobilization (long-term immobilization due to serious illness, after surgery, etc.);
- erroneous diagnosis with the use of calcium and/or vitamin D supplements;
- erroneous prescription of antacid drugs for duodenal ulcers caused by hyperparathyroidism;
- treatment with thiazide diuretics without taking into account contraindications;
- eating foods rich in calcium and phosphorus (milk, nutritional supplements, as well as foods containing increased amounts of vitamin D).
Intense secretion characteristic of hyperparathyroidism gastric juice against the background of extreme hypercalcemia can lead to the formation of multiple ulcers and the development of gastrointestinal bleeding.
Developing quickly high fever with an increase in temperature to 39-40 degrees and higher. Muscle weakness, decreased tendon reflexes, and bone pain are observed. The skin becomes dry, itching appears, which often leads to scratching.
Various psychoneurological disorders are on the rise, from depression to severe psychomotor agitation (up to psychosis). As the pathology progresses, consciousness becomes confused, and the patient falls into coma.
In the future, due to the activation of blood coagulation factors, DIC syndrome may develop. With extremely high levels of hypercalcemia, shock may develop. Death usually occurs due to paralysis respiratory center and/or cardiac arrest.
Hyperparathyroidism in children
Primary hyperparathyroidism in children, especially under the age of 10 years, is extremely rare. According to statistics, girls get sick somewhat more often than boys. Just as in adults, the most common cause of hyperparathyroidism in children is a single benign tumor (adenoma) of the parathyroid gland. Glandular hyperplasia is much less common.In newborns, hereditary hyperparathyroidism occurs due to genetic defect receptors of parathyroid cells for calcium. The mechanism of development of this pathology is similar to secondary hyperparathyroidism - the parathyroid glands undergo working hyperplasia, since defective receptors register a reduced level of calcium.
There are two forms of this hereditary disease:
1.
Severe homozygous - when pathological genes are received from both parents.
2.
More benign is heterozygous, when the influence of the pathological gene is somewhat balanced by the normal gene.
The most common cause of secondary hyperparathyroidism in children, as well as in adults, is severe renal failure or malabsorption syndrome.
In children younger age the secondary type of the disease often develops with rickets and rickets-like diseases.
Hyperparathyroidism developed in early age, causes a lag in physical and mental development.
Treatment for children is the same as for adults.
Diagnostics
Primary hyperparathyroidism
P
Since the initial stages of hyperparathyroidism are often asymptomatic, laboratory methods are of paramount importance in diagnosing the disease. Primary hyperparathyroidism is characterized by: laboratory symptoms, How:
- increased levels of calcium in the blood;
- decreased level of phosphates in blood plasma;
- increased excretion of calcium in the urine;
- increased excretion of phosphates in the urine.
If an elevated level of parathyroid hormone confirms the diagnosis of hyperparathyroidism, topical diagnosis is performed. For this purpose, ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI) of the head and neck area are used.
Additionally, complications of hyperparathyroidism (osteoporosis, kidney damage) are diagnosed.
When diagnosing primary hyperparathyroidism, it is necessary to carry out a differential diagnosis with diseases that occur with elevated levels of calcium in the blood:
- malignant tumors (bone metastases, multiple myeloma);
- hypervitaminosis D;
- secondary hyperparathyroidism;
- rare causes (thyrotoxicosis, taking thiazide diuretics, hypervitaminosis A, Addison's disease, etc.).
Secondary hyperparathyroidism
Secondary hyperparathyroidism is characterized by an increased level of parathyroid hormone with a normal calcium concentration in the blood plasma.Determination of the level of parathyroid hormone is indicated for any kidney pathology, accompanied by a decrease in the rate of glomerular filtration up to 60% and below.
Topical diagnosis and diagnosis of complications are carried out according to the same rules as for the primary type of disease.
Differential diagnosis is primarily carried out between diseases that can cause secondary hyperparathyroidism. Therefore, the examination is always supplemented by the study of the pathology that led to prolonged hypocalcemia, which caused increased production of parathyroid hormone.
Tertiary hyperparathyroidism
This type of disease is characterized by an increase in the level of parathyroid hormone in the blood by 10-25 times with normal calcium levels. This diagnosis is made when secondary hyperparathyroidism is resistant to conservative treatment and hypercalcemia occurs.Treatment
Hypercalcemic crisis
Relief from a hypercalcemic crisis is carried out in the endocrinology or intensive care department. All therapeutic measures are aimed at quickly reducing calcium levels in the blood. First of all, under the control of the electrolyte composition of the blood, forced diuresis is carried out: 3.0 liters of isotonic sodium chloride solution is injected intravenously over three hours, in combination with the administration of the diuretic furosemide 100 mg/hour.
In addition, free calcium in the blood is bound using Complexon - a 5% solution sodium salt ethylenediaminetetraacetic acid (Na 2 -EDTA). Over 5-6 hours, a dose equal to 50 mg/kg of the patient’s body weight is administered.
Finally, calcium is fixed in the bones using Calcitrin (1-4 IU/kg of the patient’s body weight).
To accelerate the removal of calcium from the body, extracorporeal methods (blood purification outside the body) such as hemodialysis and peritoneal dialysis with calcium-free dialysate can be used.
Primary hyperparathyroidism
Practice has shown that the only effective method The treatment for primary hyperparathyroidism is surgical.However, the initial stages of hyperparathyroidism most often occur without pronounced symptoms, and the preclinical period of the disease is 10 years or more. Considering the advanced age of most patients (pathology most often develops in older men and women during menopause), attention is paid to the indications for surgery, which are divided into absolute and relative.
Absolute indications for surgical treatment:
- blood calcium level more than 3 mmol/l;
- episodes of hypercalcemia in the past;
- severe renal dysfunction;
- stones in the upper urinary tract (even if there are no symptoms of urolithiasis);
- excretion of calcium in the urine more than 10 mmol per day;
- severe osteoporosis.
- severe concomitant diseases;
- complexity of dynamic observation;
- young age (up to 50 years);
- patient's wishes.
In cases where we are talking about diffuse hyperplasia of the parathyroid glands, a subtotal parathyroidectomy is performed - three glands and part of the fourth are removed, leaving an area that is sufficiently well supplied with blood. Relapses after such an operation occur in 5% of cases.
Patients for whom surgery is not indicated are prescribed constant monitoring:
- constant monitoring of blood pressure and calcium levels in the blood;
- kidney function testing every 6-12 months;
- Carrying out bone densitometry and ultrasound of the kidneys once every two to three years.
Secondary hyperparathyroidism
Drug treatment of secondary hyperparathyroidism consists of prescribing vitamin D supplements, and if there is a tendency to hypocalcemia, in combination with calcium supplements (up to 1 g/day).The indication for subtotal parathyroidectomy is the failure of the ongoing conservative treatment. Surgery is resorted to when the level of parathyroid hormone in the blood plasma increases three times or more, as well as when hypercalcemia is 2.6 mmol/l or more.
Tertiary hyperparathyroidism
The term "tertiary hyperparathyroidism" usually refers to a state of increased production of parathyroid hormone in patients after kidney transplantation.It should be noted that the reverse involution of hyperplastic parathyroid glands takes time - months, and sometimes even years. However, if high levels of parathyroid hormone and calcium in the blood do not decrease despite therapy with Calcitriol, and there is a real threat of complications, subtotal parathyroidectomy is indicated.
Hyperparathyroidism: description, diagnosis, treatment - video
Forecast
With timely diagnosis of primary hyperparathyroidism and successful removal of a tumor producing parathyroid hormone, the prognosis is favorable. Restoration of bone tissue structure occurs, as a rule, during the first two years after surgery. Pathological symptoms hyperparathyroidism on the part of the nervous system and internal organs disappear within a few weeks. parathyroid hormone. Increased hormone production is a consequence glandular hyperplasia
, which in turn leads to disruption of phosphorus calcium metabolism. This results in increased removal of phosphorus and calcium from the skeleton, an increase in osteoclastic processes and their excessive entry into the blood in large quantities. Simultaneous increased phosphorus release, as well as decreased tubular reabsorption leads to the emergence hypophosphatemia And hyperphosphaturia , at the same time, signs and osteomalacia . Most often, 2-3 times more than men, the disease affects women aged 25 to 50 years.
Hyperparathyroidism is caused by a tumor of the parathyroid glands.
Depending on the cause of its occurrence, hyperparathyroidism is divided into the following types:
- Primary appears as a consequence of the formation of parathyroid adenoma in the vast majority of cases of the disease. And only in one out of ten cases of disease is the cause carcinomas or hyperplasia, proliferation and enlargement of normal gland cells.
- Secondary hyperparathyroidism- there is an increase in function, pathological growth and enlargement of the glands, a long-term low calcium content with a simultaneous increased phosphate content in the blood. There is an increase in production parathyroid hormone for chronic renal failure.
- Tertiary- development of benign tumors of the parathyroid glands is observed, as well as increased production parathyroid hormone due to long-term secondary hyperparathyroidism.
- Pseudohyperparathyroidism– production of parathyroid hormone is observed in tumors that did not arise from the cells of the parathyroid glands.
According to severity, the disease is divided into
- manifesto form.
- asymptomatic (soft) form.
- asymptomatic form.
In addition, according to the degree of disease, the disease is divided into bone , renal , visceral And mixed forms.
Symptoms of hyperparathyroidism
The danger of the disease is that it can occur without symptoms and the discovery or diagnosis of hyperparathyroidism occurs by chance during examination. In the early stages of the disease, the patient develops rapid fatigue even with light loads, difficulty walking, and especially when climbing stairs, a characteristic waddling “duck” gait.
Patients experience emotional imbalance, resentment and anxiety, memory deteriorates, and depression appears. The skin takes on an earthy gray color. In old age, various symptoms may appear.
Subsequently, signs of damage to various internal organs develop - cholelithiasis, osteoporosis, etc.
The late stage of bone hyperparathyroidism is characterized by softening and curvature of the bones, the appearance of scattered pain in the bones of the arms or legs, and in the spine. Normal movements can lead to bone fractures, which are not painful, but heal slowly, sometimes causing false joints.
Because of deformed skeleton, the patient may even become shorter. With osteoporosis, the patient's jaws become loose or fall out. healthy teeth. A large one is felt on the neck in the area of the parathyroid glands. Visible signs appear on the limbs periarticular calcifications .
At visceropathic hyperparathyroidism Nausea, vomiting, and sudden weight loss are observed. Patients complain of loss, stomach pain, flatulence. The examination reveals the appearance of peptic ulcers with, as well as various signs damage to the pancreas and gallbladder, polyuria and symptoms of renal failure develop. The nutrition of organs and tissues is disrupted, a high concentration of calcium in the blood causes damage to the heart vessels, increased blood pressure, . With calcification of the ocular conjunctiva, the so-called “red eye” syndrome is observed.
At renal form main symptoms of hyperparathyroidism: polyuria and alkaline urine reaction. Possible development of bilateral nephrocapcinosis , which, in turn, can lead to and uremia . The patient is concerned about high blood pressure, seizures renal colic, dyspeptic disorders. An ulcer of the duodenum or stomach appears, and perforation of the wall of the stomach and intestines is possible. Often possible chronic , formation of gallstones.
Diagnosis of hyperparathyroidism
Diagnosis of the disease is carried out on the basis of blood tests that determine calcium and phosphorus in the body and urine analysis.
If high calcium levels are detected, other tests and studies are performed: ultrasonography, X-ray examination, CT and MRI, which can detect osteoporosis, pathological ulcers of the gastrointestinal tract, cystic bone changes and other changes. Scintigraphy parathyroid glands reveals the localization of the glands and their anomaly.
In secondary hyperparathyroidism, the underlying disease is diagnosed.
The doctors
Treatment of hyperparathyroidism
Treatment of the disease is carried out comprehensively in combination with conservative therapy medications and operating surgery. Before surgery, conservative treatment is carried out, the goal of which is to reduce blood Ca level.
Malignant tumors of the parathyroid glands are surgically removed, then radiation therapy.
The prognosis of hyperparathyroidism is favorable with timely diagnosis of hyperparathyroidism and adequate surgical treatment. Full recovery ability to work depends on the degree of damage to bone tissue. If treatment for hyperparathyroidism is started at an early stage, the patient recovers within a maximum of six months. In moderate to severe cases, recovery lasts for 2 years. In advanced cases, disability is likely.
The prognosis for renal forms of hyperparathyroidism is less favorable and depends entirely on the degree of the kidneys to surgical intervention. Without surgery - disability and death due to progressive cachexia and chronic renal failure.
At hypercalcemic crisis the prognosis depends on the timeliness of treatment, mortality is 32%.
List of sources
- Endocrinology. Ed. N. Lavin. - Moscow: Praktika, 1999;
- Pathophysiology of the endocrine system / ed. ON THE. Smirnova. - M.: Binom, 2009;
- Endocrinology / Dedov I.I. and others. M.: Medicine, 2007.
Hyperparathyroidism – chronic pathology parathyroid glands, progressing due to the occurrence of tumors or increased proliferation of their tissues. The pathology is characterized by increased production of parathyroid hormone, which affects calcium metabolism. Its excess content in the blood causes calcium to be washed out of the bones, and this, in turn, leads to serious complications.
Among all endocrine diseases, hyperparathyroidism ranks 3rd - only and are more common than this disease. Women suffer from this pathology several times more often than men (especially women over 45 years of age). Hyperparathyroidism sometimes occurs in children due to congenital pathology of the parathyroid glands.
Causes
The disease occurs when the parathyroid glands produce a large number of parathyroid hormone, which leads to an increase in calcium concentration in the blood and a decrease in phosphorus levels. Everyone knows that calcium plays an important role in our body. It is necessary for normal functioning bone skeleton, and is also an important component of teeth. In addition, with its help, signals are transmitted to the muscles to ensure their functioning.
The reasons for increased calcium levels in the blood can be hereditary and physiological. Hereditary causes- This congenital pathologies in the structure, quantity and function of the parathyroid glands. However, such reasons are less common than physiological ones, that is, those that arise as a result of the vital activity of the body.
The main causes of hyperparathyroidism are the following:
- lack of vitamin D;
- tumors of various origins on the parathyroid glands;
- intestinal diseases in which absorption processes in the organ are disrupted;
- hyperplasia of two or more parathyroid glands.
Depending on the cause, there are several types of this pathology. So, in medical practice There are primary, secondary and tertiary hyperparathyroidism. In addition, there is the so-called nutritional and pseudohyperparathyroidism.
Primary is caused by the direct pathology of this organ. Most often, this disease occurs when the organ has benign adenoma, and less often (5% of cases) the cause is multiple tumors. Very rarely, primary hyperparathyroidism occurs as a consequence of a cancerous tumor on the parathyroid gland.
This type of disease is also caused by hyperplasia of the parathyroid glands.
Secondary hyperparathyroidism occurs as a consequence of calcium metabolism disorders due to other pathological disorders in the human body. Due to the lack of this microelement in the blood, due to some internal disorders, the parathyroid glands begin to actively produce calcitonin, which leads to the development.
Most often, secondary hyperparathyroidism develops as a result of complex pathologies of internal organs, for example, kidney pathology and pathological disorders in the intestines. Respectively this variety The disease can be of two forms:
- renal;
- interstinal.
A pathology such as nutritional hyperparathyroidism, refers to the secondary form and it occurs against the background of malnutrition. The disease occurs when the diet of children or adults is unbalanced and there is a lack of calcium. Treatment of hyperparathyroidism of this form is the simplest - it is necessary to normalize the amount of calcium entering the body with food.
Tertiary hyperparathyroidism is a fairly rare form of the disease, which causes a disruption in the production of parathyroid hormone due to kidney transplantation.
The last type is pseudohyperparathyroidism. This is a condition that occurs with certain cancers, such as breast and lung tumors. Often this type pathological disorder observed due to the fact that cancer cells are capable of producing substances with properties similar to parathyroid hormone.
Symptoms
Very often, at the beginning of its development, the disease is asymptomatic, so its diagnosis is difficult. There is only one sign of illness - an increased level of calcium in the blood, which is detected by chance during a patient's examination for other health problems or during a medical examination.
In most people, at the initial stage, disorders are observed only general symptoms, such as:
- Bad mood;
- fatigue;
- decreased appetite;
- the appearance of constipation;
- painful sensations in bones and joints.
Sometimes unreasonable nausea and even vomiting may occur.
As the disease progresses, the symptoms become more pronounced and are associated with impaired neuromuscular conduction. There is weakness in the muscles, painful sensations in them, the occurrence of difficulties when walking. To get up from a chair, a person with such a disorder requires support, and he cannot independently climb the stairs, get on a tram, etc. without assistance.
Other symptoms this violation– this is looseness of the joints and the development of a “duck gait”. Because of this disorder, people may become bedridden before the bones become brittle and pathological fractures occur. It is the fragility of bones and the occurrence permanent fractures– these are important symptoms of the progression of the pathology. A person also loses teeth, even those that were completely healthy.
In addition to those described above, other symptoms are also observed with hyperparathyroidism, for example, a sharp decrease in body weight, development leading to dehydration, earthy skin tone and dryness, development. A person with this disorder looks gaunt and tired.
In severe cases of the disease, disorders of the internal organs are noted. In particular, the following symptoms may occur:
- skeletal damage (severe bone deformities, and pathological fractures, false and true gout);
- development, the nature of which is irreversible;
- disruption of the gastrointestinal tract, resulting in the development of symptoms such as pain, nausea, vomiting, diarrhea, etc.;
- development and
If we talk about secondary and tertiary hyperparathyroidism, their symptoms are usually associated with musculoskeletal system. Characteristic is eye damage, manifested by persistent conjunctivitis.
One of the most difficult, although rare complications This pathology is a hypercalcemic crisis, which is characterized by:
- development of cardiovascular failure;
- increased blood clotting;
- severe disturbances of nervous activity.
This pathological condition can cause or lead to cardiac arrest, so when a hypercalcemic crisis develops, the mortality rate is about 60%.
Diagnostics
Diagnosis of the disease at an early stage is important in the treatment of pathology. The main importance in diagnosing the disease is given to laboratory research methods, which make it possible to determine the increased calcium content in the blood and insufficient phosphorus content.
Diagnosis also involves ultrasound of the parathyroid glands, CT and MRI.
When diagnosing primary hyperparathyroidism, it is necessary differential diagnosis this disease, with pathologies such as hypervitaminosis D or malignant tumors in the body.
Diagnosis of other forms of this disorder is carried out in a similar way. In addition, patients with any type of this pathology need diagnosis of complications that could develop against its background.
Treatment
When the form of pathology is established, its treatment will depend on the data obtained during diagnosis. To relieve a hypercalcemic crisis, the patient is placed in the intensive care unit and undergoes forced diuresis. Hemodialysis and other emergency measures are also indicated.
Treatment of hyperparathyroidism is mainly carried out surgically. Surgical treatment consists of removing the tumor on the gland, or removing excess (overgrown) tissue. The secondary form can be treated with medication - for this purpose, vitamin D preparations are prescribed. If conservative treatment does not have an effect, subtotal parathyroidectomy is indicated.
Hyperparathyroidism is a pathological condition of the parathyroid glands associated with disruption of their functioning and the production of excessive amounts of parathyroid hormone. This leads to the release of calcium from bone tissue, an increase in its concentration in the blood and accumulation in other organs, usually in the kidneys. Hyperparathyroidism is more common in adults; in children, the pathology is observed much less frequently. According to medical statistics, in families where there are patients with hyperparathyroidism, in 30% of cases the disease manifests itself in children.
The parathyroid glands are located on the posterior surface of the thyroid gland. Their increased functioning leads to an excess of parathyroid hormone in the blood and its accumulation in the internal organs.
Increased function of the parathyroid glands, observed in childhood, can have various reasons:
- idiopathic hyperplasia (increase in the size and function of the glands for an unknown reason);
- rickets;
- thyroid adenoma;
- chronic disorder of the processes of digestion and absorption of nutrients in small intestine;
- sarcoidosis;
- increased blood phosphate concentrations in chronic kidney disease (hyperphosphatemia);
- tuberculosis;
- hypervitaminosis D;
- Williams syndrome;
- necrosis of subcutaneous fat in newborns;
- oncological diseases (leukemia, neuroblastoma, dysgerminoma, etc.);
- genetic predisposition (familial benign hyperparathyroidism), etc.
The development of hyperparathyroidism in children can be triggered by:
- uncontrolled reception hormonal drugs and antibiotics;
- unfavorable environment;
- eating carcinogenic foods, etc.
Symptoms
The clinical picture of hyperparathyroidism in children is similar to that of adults. Signs of the disease:
- irritability;
- drowsiness;
- weakness;
- malaise;
- problems with weight gain;
- muscle weakness;
- nausea and vomiting;
- constipation;
- fever;
- mental disorders;
- delay physical development and etc.
Due to the fact that calcium is excreted into the blood in excess quantities, it is deposited in the renal parenchyma; this leads to the development of hematuria and renal colic. Calcium leaching is accompanied by a pathological change in the structure of bone tissue.
Loose teeth or late eruption are noted. Children complain of pain in the back, stomach and limbs. Patients with hyperparathyroidism may experience hallux valgus knee joints, compression fractures vertebrae, gait disturbances, etc. Long-term hypercalcemia leads to seizures, mental retardation and blindness.
Diagnostics
Secondary hyperparathyroidism is most often discovered incidentally. Primary pathology is diagnosed using the following studies:
- Analysis of urine;
- blood chemistry;
- biopsy and histological examination of bone tissue;
- densitometry (determination of bone density);
- MRI and CT of the head and neck;
- Ultrasound of the thyroid gland along with parathyroid glands, etc.
How to treat
To correct blood chemical parameters, the child is given forced diuresis. Treatment of hyperparathyroidism in children is complex. Depending on the child’s condition and the stage of development of the disease, one or more treatment methods are used:
- First of all, the patient is given forced diuresis to adjust blood chemical parameters. Up to 3.5 liters of isotonic sodium chloride solution is injected intravenously over several hours.
- Taking vitamin D supplements in a therapeutic dose.
- Chemotherapy is treatment with chemotherapy drugs.
- Surgical intervention (most often reduced to subtotal parathyroidectomy). Patients undergo partial or complete removal of the adenoma, if any, damaged parathyroid glands and adjacent tissues. Currently, such operations are performed using a minimally invasive, endoscopic method.
- Taking hormonal medications - corticosteroids, bisphosphonates, calcitonin. Hormone therapy helps inhibit the release of calcium from the skeletal system.
- Treatment radioactive iodine and etc.
During the treatment of hyperparathyroidism in children, stop taking vitamins containing calcium and medicines, helping to increase its level in the body. In addition, they appoint additional treatment drugs that stop muscle and bone degeneration.
At early diagnosis and timely surgical treatment, the prognosis is favorable. However, severe and extensive bone deformities can last a lifetime.
Nutrition for hyperparathyroidism
Proper nutrition for hyperparathyroidism is the key to stabilizing the patient’s condition and speedy recovery. Children with this endocrine pathology must strictly follow a diet. Parents should:
- exclude from the child’s diet or limit to a minimum foods with high content calcium (dairy and milk); babies with a congenital form of pathology are switched from eating soy mixtures;
- increase in the patient’s menu the amount of foods rich in phosphorus,) and vitamin D ( butter, cheese);
- ensure that the child drinks (1-2 liters per day depending on age);
- use in cooking iodized salt or salt with reduced content sodium;
- completely eliminate “harmful” foods rich in carcinogens, preservatives, salt, fats, dyes.
Summary for parents
A child suffering from hyperparathyroidism should drink sufficient quantity water. To prevent the development of hyperparathyroidism in children, it is necessary to ensure that the child’s diet contains foods containing
– endocrinopathy, which is based on excessive production of parathyroid hormone by the parathyroid glands. Hyperparathyroidism leads to increased calcium levels in the blood and pathological changes that occur primarily in bone tissue and kidneys. The incidence of hyperparathyroidism among women is 2–3 times more common than among men. Women between 25 and 50 years of age are more susceptible to hyperparathyroidism. Hyperparathyroidism can have a subclinical course, bone, visceropathic, mixed form, and acute course in the form of a hypercalcemic crisis. Diagnosis includes determination of Ca, P and parathyroid hormone in the blood, x-ray examination and densitometry.
General information
– endocrinopathy, which is based on excessive production of parathyroid hormone by the parathyroid glands. Hyperparathyroidism leads to increased calcium levels in the blood and pathological changes that occur primarily in bone tissue and kidneys. The incidence of hyperparathyroidism among women is 2–3 times more common than among men. Women between 25 and 50 years of age are more susceptible to hyperparathyroidism.
Classification and causes of hyperparathyroidism
Hyperparathyroidism can be primary, secondary and tertiary. Clinical forms Primary hyperparathyroidism can be varied.
Primary hyperparathyroidism
Primary hyperparathyroidism is divided into three types:
I. Subclinical primary hyperparathyroidism.
- biochemical stage;
- asymptomatic stage (“silent” form).
II. Clinical primary hyperparathyroidism. Depending on the nature of the most pronounced symptoms, the following are distinguished:
- bone form (parathyroid osteodystrophy, or Recklinghausen's disease). It manifests itself as deformation of the limbs, leading to subsequent disability. Fractures appear “on their own”, without injury, heal long and difficult, and a decrease in bone density leads to the development of osteoporosis.
- visceropathic form:
- renal - with a predominance of severe urolithiasis, with frequent attacks renal colic, development of renal failure;
- gastrointestinal form - with manifestations of stomach and duodenal ulcers, cholecystitis, pancreatitis;
- mixed form.
III. Acute primary hyperparathyroidism(or hypercalcemic crisis).
Primary hyperparathyroidism develops when the parathyroid glands contain:
- one or more adenomas (benign tumor-like formations);
- diffuse hyperplasia (increase in the size of the gland);
- hormonally active cancer (rarely, in 1-1.5% of cases).
In 10% of patients, hyperparathyroidism is combined with various hormonal tumors(pituitary tumors, thyroid cancer, pheochromocytoma). Primary hyperparathyroidism also includes hereditary hyperparathyroidism, which is accompanied by other hereditary endocrinopathies.
Secondary hyperparathyroidism
Secondary hyperparathyroidism serves as a compensatory reaction to long-term low levels of Ca in the blood. In this case, increased synthesis of parathyroid hormone is associated with impaired calcium-phosphorus metabolism in chronic renal failure, vitamin D deficiency, malabsorption syndrome (impaired absorption of Ca in the small intestine). Tertiary hyperparathyroidism develops in the case of untreated long-term secondary hyperparathyroidism and is associated with the development of an autonomously functioning parathyroid adenoma.
Pseudohyperparathyroidism (or ectopic hyperparathyroidism) occurs with malignant tumors of various localizations (breast cancer, bronchogenic cancer), capable of producing parathyroid hormone-like substance, with multiple endocrine adenomatosis types I and II.
Hyperparathyroidism is manifested by an excess of parathyroid hormone, which promotes the removal of calcium and phosphorus from bone tissue. Bones become weak, soften, can bend, and the risk of fractures increases. Hypercalcemia (excessive levels of Ca in the blood) leads to the development of muscle weakness and the excretion of excess Ca in the urine. Urination increases, constant thirst, is developing kidney stones(nephrolithiasis), deposition of calcium salts in the kidney parenchyma (nephrocalcinosis). Arterial hypertension in hyperparathyroidism is due to the effect of excess Ca on the tone of blood vessels.
Symptoms of hyperparathyroidism
Hyperparathyroidism can be asymptomatic and diagnosed accidentally during examination. With hyperparathyroidism, the patient simultaneously develops symptoms of the lesion various organs and systems - stomach ulcers, osteoporosis, urolithiasis, cholelithiasis, etc.
TO early manifestations hyperparathyroidism include rapid fatigue during exercise, muscle weakness, headache, difficulty walking (especially when climbing or covering long distances), and a waddling gait is characteristic. Most patients report memory impairment, emotional imbalance, anxiety, and depression. Older people may experience severe mental disorders. With long-term hyperparathyroidism, the skin becomes an earthy gray color.
At the late stage of bone hyperparathyroidism, softening, curvature, pathological fractures (during normal movements, in bed) of the bones occur, and scattered pain occurs in the bones of the arms and legs, and the spine. As a result of osteoporosis, the jaws become loose and healthy teeth fall out. Due to skeletal deformation, the patient may become shorter. Pathological fractures are not painful, but heal very slowly, often with deformities of the limbs and the formation of false joints. Periarticular calcifications are found on the arms and legs. A large adenoma can be palpated on the neck in the area of the parathyroid glands.
Visceropathic hyperparathyroidism is characterized by nonspecific symptoms and a gradual onset. With the development of hyperparathyroidism, nausea, stomach pain, vomiting, flatulence occur, appetite is impaired, and weight decreases sharply. Patients are found peptic ulcers with bleeding various localizations, prone to frequent exacerbations, relapses, as well as signs of damage to the gallbladder and pancreas. Polyuria develops, urine density decreases, and unquenchable thirst appears. On late stages Nephrocalcinosis is detected, symptoms of renal failure develop, progressing over time, and uremia.
Hypercalciuria and hypercalcemia, the development of calcification and vascular sclerosis, leads to impaired nutrition of tissues and organs. A high concentration of Ca in the blood contributes to damage to the heart vessels and increased blood pressure, causing angina attacks. When the conjunctiva and cornea of the eyes become calcified, red eye syndrome occurs.
Complications of hyperparathyroidism
Hypercalcemic crisis refers to severe complications of hyperparathyroidism that threaten the patient's life. Risk factors include long-term bed rest, uncontrolled intake of Ca and vitamin D supplements, thiazide diuretics (reduce Ca excretion in the urine). A crisis occurs suddenly with acute hypercalcemia (Ca in the blood is 3.5 - 5 mmol/l, when the norm is 2.15 - 2.50 mmol/l) and is manifested by a sharp exacerbation of all clinical symptoms. This condition is characterized by: high (up to 39 – 40°C) body temperature, acute epigastric pain, vomiting, drowsiness, impaired consciousness, coma. Weakness increases sharply, dehydration occurs, especially severe complication– development of myopathy (muscle atrophy) of the intercostal muscles and diaphragm, proximal parts of the body. Pulmonary edema, thrombosis, bleeding, and perforation of peptic ulcers may also occur.
Diagnosis of hyperparathyroidism
Primary hyperparathyroidism does not have specific manifestations, so diagnosis should be made by clinical picture pretty hard. It is necessary to consult an endocrinologist, examine the patient and interpret the results obtained:
- general urine test
Urine acquires alkaline reaction, the excretion of calcium in the urine (hypercalciuria) and an increase in the content of P in it (hyperphosphaturia) are determined. The relative density drops to 1000, and there is often protein in the urine (proteinuria). Granular and hyaline casts are found in the sediment.
- biochemical blood test (calcium, phosphorus, parathyroid hormone)
The concentration of total and ionized Ca in the blood plasma increases, the P content is below normal, activity alkaline phosphatase increased. More indicative of hyperparathyroidism is the determination of the concentration of parathyroid hormone in the blood (5-8 ng/ml and higher, with a norm of 0.15-1 ng/ml).
- ultrasound examination
Ultrasound of the thyroid gland is informative only when parathyroid adenomas are located in typical places- in the area of the thyroid gland.
- x-ray examination, CT and MRI
X-rays can detect osteoporosis, cystic bone changes, and pathological fractures. Densitometry is performed to assess bone density. Using an X-ray examination with a contrast agent, peptic ulcers in the gastrointestinal tract that occur with hyperparathyroidism are diagnosed. CT scan of the kidneys and urinary tract reveals stones. X-ray tomography of the retrosternal space with esophageal contrast with a barium suspension makes it possible to identify parathyroid adenoma and its location. Magnetic resonance imaging is more informative than CT and ultrasound and visualizes any location of the parathyroid glands.
- scintigraphy of the parathyroid glands
Allows you to identify the localization of usually and abnormally located glands. In the case of secondary hyperparathyroidism, the underlying disease is diagnosed.
Treatment of hyperparathyroidism
Complex treatment of hyperparathyroidism combines surgery and conservative therapy medications. The main treatment for primary hyperparathyroidism is surgery, which involves removing parathyroid adenoma or hyperplastic parathyroid glands. Today, surgical endocrinology has minimally invasive methods of surgical interventions performed for hyperparathyroidism, including the use of endoscopic equipment.
If the patient has been diagnosed with a hypercalcemic crisis, surgery is necessary to emergency indications. Before surgery, it is necessary to prescribe conservative treatment aimed at reducing Ca in the blood: drinking plenty of fluids, intravenous - isotonic NaCl solution, in the absence of renal failure - furosemide with KCl and 5% glucose, extract of the thyroid glands of cattle (under the control of Ca levels in the blood), bisphosphonates (pamidronic acid and sodium etidronate), glucocorticoids.
After surgery for malignant tumors of the parathyroid glands, radiation therapy is performed, and an antitumor antibiotic, plicamycin, is also used. After surgical treatment, most patients have a decrease in the amount of Ca in the blood, so they are prescribed vitamin D supplements (in more severe cases, Ca salts intravenously).
Forecast and prevention of hyperparathyroidism
The prognosis of hyperparathyroidism is favorable only in case of early diagnosis and timely surgical treatment. Restoring the patient's normal working capacity after surgical treatment Bone hyperparathyroidism depends on the degree of bone tissue damage. At mild flow disease, performance is restored after surgical treatment within approximately 3-4 months, in severe cases - within the first 2 years. In advanced cases, work-limiting bone deformities may remain.
In the renal form of hyperparathyroidism, the prognosis for recovery is less favorable and depends on the severity of kidney damage at the preoperative stage. Without surgical intervention, patients usually become disabled and die from progressive cachexia and chronic renal failure. With the development of a hypercalcemic crisis, the prognosis is determined by the timeliness and adequacy of the treatment; the mortality rate for this complication of hyperparathyroidism is 32%.
In case of existing chronic renal failure, it is important drug prophylaxis secondary hyperparathyroidism.
