Primary hyperparathyroidism is a severe endocrine disease characterized by increased secretion of the parathyroid glands. Pathology affects primarily the bone apparatus and kidneys. What are the causes of this violation? And how to recognize the initial signs of the disease? We will answer these questions in the article.
Pathology Description
On the posterior surface of the thyroid gland are two pairs of parathyroid glands. They produce parathyroid hormone (PTH). This substance is responsible for the metabolism of calcium and phosphorus. PTH has the following effects on the body:
- Promotes the release of calcium from the bones and increase its concentration in the blood.
- Enhances the excretion of phosphorus in the urine.
If the hormone PTH is produced in increased quantities, then doctors call this condition hyperparathyroidism. This violation may be due to various reasons. If the increased production of parathyroid hormone is caused by pathological changes in the parathyroid glands (tumor or hyperplasia), then experts are talking about primary hyperparathyroidism. If this endocrine disorder is provoked by diseases of other organs (most often the kidneys), then it is secondary in nature.
The enhanced production of parathyroid hormone adversely affects the whole body, and especially on the skeletal system and kidneys. Increased secretion of PTH leads to the excretion of calcium from bones and an increase in its concentration in plasma (hypercalcemia). This causes the following systemic disorders:
- the formation of fibrotic changes in the bones;
- skeletal deformations;
- calcium deposits in the kidneys and on the walls of blood vessels;
- slowing down the transmission of nerve impulses;
- arterial hypertension;
- increased secretion of gastric juice;
- the appearance of ulcers in the digestive tract.
In addition, the patient dramatically increases the excretion of phosphorus through the kidneys. This leads to the appearance of stones in the organs of excretion.
Primary hyperparathyroidism is much more common in women than in men. Often, signs of the disease appear during menopause. This pathology is also called parathyroid osteodystrophy or Engel-Recklinghausen disease. This endocrine disorder is quite common. It ranks third in prevalence after diabetes and thyroid dysfunction.
Causes
The causes of primary hyperparathyroidism are the following pathological changes in the parathyroid glands:
- adenoma;
- hyperplasia
- malignant tumor.
In 90% of cases, hyperparathyroidism develops due to the formation of an adenoma on one or more parathyroid glands. This benign tumor is most often diagnosed in elderly patients, especially in postmenopausal women.
Much less often, the cause of hyperparathyroidism is the proliferation of tissue (hyperplasia) of the glands. This pathology usually has a hereditary character and is noted in young patients. Hyperplasia is often accompanied by disorders of the function and other endocrine organs.
Parathyroid cancer is extremely rare, in only 1-2% of cases. Malignant tumors form after radiation exposure of the neck or head.
It is important to remember that approximately 15-20% of people have additional parathyroid glands located in the mediastinum. This is a variant of the norm. However, additional organs can also undergo pathological changes. There are cases when the glands in the neck are completely healthy, but the patient has increased PTH hormone. This may indicate a tumor or hyperplasia in additional organs, which greatly complicates the diagnosis.
Varieties of pathology
As already mentioned, increased production of parathyroid hormone negatively affects the functions of different organs. Depending on the location of the lesion and symptoms, doctors distinguish the following forms of primary hyperparathyroidism:
- Bone. In this type of pathology, severe damage to the musculoskeletal system is noted. Bones become extremely fragile and deformed. Patients suffer from frequent injuries. Fractures occur even in the absence of bruises and grow together for a very long time.
- Visceral. With this form of pathology, mainly internal organs are affected. Due to hypercalcemia, patients develop kidney stones and gall bladder, there are signs of atherosclerosis. In addition, ulcers in the gastrointestinal tract appear, vision deteriorates, and the neuropsychic sphere suffers. Pathological changes in bone tissue are weakly expressed.
- Mixed. In patients, damage to the bones and internal organs due to hypercalcemia is simultaneously noted.
ICD: classification of pathology
ICD-10 primary hyperparathyroidism is considered as a dysfunction of the parathyroid glands. This class of diseases is indicated by the code E21. This group of pathologies includes all endocrine disorders, accompanied by an increase in the secretion of parathyroid hormone. The complete code for primary hyperparathyroidism according to ICD-10 is E21.0.
Initial symptoms
At an early stage, the disease can proceed without severe symptoms. At the beginning of the pathology, the secretion of PTH is slightly increased. As a result, dysfunction of the parathyroid glands is detected late, when the patient already has a serious lesion of the bones and internal organs. You can diagnose the disease at an early stage only with the help of a blood test for hormones.
The first signs of endocrine disorder appear with a significant increase in the secretion of parathyroid hormone. Symptoms and treatment of primary hyperparathyroidism in women and men depend on the form of the disease. However, we can distinguish common initial signs of pathology:
- Fatigue and muscle weakness. An increased concentration of calcium leads to muscle weakness. Patients get tired quickly, have difficulty walking for a long time. It is often difficult for patients to get up from a chair without support or go through the door of public transport.
- Musculoskeletal pain. This is the initial sign of leaching of calcium from tissues. Most often, pain in the feet occurs. A duck walk is characteristic . Due to the pain syndrome, patients walk overwhelmingly.
- Frequent urination and thirst. In patients with primary hyperparathyroidism, urinary excretion of calcium increases. This leads to damage to the renal tubules. The tissues of the excretory organ lose their sensitivity to the pituitary hormone - vasopressin, which regulates diuresis.
- Deterioration of teeth. This early manifestation of pathology is associated with calcium deficiency. Often the first sign of illness is staggering and tooth loss, as well as rapidly progressive caries.
- Slimming, discoloration of the skin. The weight of patients in the first months of the disease may decrease by 10 - 15 kg. Rapid diuresis leads to severe dehydration, which becomes the cause of weight loss. The skin of patients becomes excessively dry and acquires a grayish or earthy hue.
- Neuropsychic disorders. Hypercalcemia leads to a deterioration in brain tissue. Patients have frequent headaches, mood swings, increased anxiety and depression.
Patients do not always associate such symptoms with endocrine disorders. Therefore, a visit to a doctor is often delayed.
At the advanced stage of pathology, the clinic of primary hyperparathyroidism is characterized by severe damage to bone tissue, blood vessels and internal organs. Increased secretion of parathyroid hormone leads to a sharp deterioration in the patient's condition. Next, we will consider in detail the pathological manifestations of various organs and systems.
Bone
Primary hyperparathyroidism is characterized by pathological changes in bone tissue. The following signs of damage to the musculoskeletal system are noted:
- Decreased bone density. The leaching of calcium and phosphorus leads to rarefaction and fragility of bone tissue (osteoporosis). Fibrosis and cysts form in the bones.
- Deformations of the skeleton. The bones become soft and easily bend. There is a curvature of the pelvis, spine, and in severe cases, and limbs. The chest becomes bell-shaped.
- Pain syndrome. Patients suffer from back and limb pain. Often there are attacks that resemble the manifestations of gout. This is due to both bone deformities and the deposition of calcium and phosphorus salts in the joints.
- Frequent fractures. Patients are injured not only in falls and bruises, but even with awkward movements. Sometimes fractures occur spontaneously when the patient is completely at rest. With hyperparathyroidism, trauma is not always accompanied by severe pain. There are times when patients do not notice fractures. In this case, healing occurs very slowly, since the bones are poorly healed.
- Growth reduction. Due to skeletal deformities, patient growth may decrease by 10 - 15 cm.
Multiple fractures can lead to disability of the patient. In advanced cases, the patient loses the ability to independently move and serve himself.
Excretory organs
With increased production of the hormone PTH, the kidneys become the second target organ after the skeletal system. Increased excretion of calcium in the urine causes damage to the tubules. In the early stages, this manifests itself in rapid urination and thirst. Over time, stones form in the organ, which is accompanied by attacks of renal colic.
The stronger the signs of kidney damage, the more unfavorable the prognosis of the disease. In severe cases, patients develop edema and develop renal failure, which is irreversible.
Vessels
Excess calcium is deposited in the walls of blood vessels. This leads to a deterioration in blood circulation and nutrition of various organs. Patients experience symptoms characteristic of cardiovascular diseases:
- headache;
- arrhythmia;
- increase in blood pressure;
- angina attacks.
Calcium deposits can also form in the myocardium, which often causes a heart attack.
Nervous system
The higher the concentration of calcium in the blood plasma, the more pronounced disorders of the nervous system and psyche. Patients complain of the following pathological manifestations:
- apathy
- lethargy;
- headache;
- dreary mood;
- anxiety;
- drowsiness;
- decreased memory and mental abilities.
In severe cases, patients experience psychotic disorders with blurred consciousness, delirium, and hallucinations.
Gastrointestinal organs
As already mentioned, the hormone PTH affects the secretion of gastric juice. Many patients with hyperparathyroidism have an increased acidity. This is accompanied by the following symptoms:
- abdominal pains of various localization;
- nausea
- increased gas formation;
- frequent constipation.
Against the background of increased acidity, ulcerative processes develop. Most often they are localized in the duodenum, less often in the stomach and esophagus. Ulcers are accompanied by frequent pain and bleeding.
Calcium salts can also be deposited in the gallbladder. This leads to inflammation of the organ (cholecystitis), and subsequently to gallstone disease. Attacks of pain in the right hypochondrium and nausea appear.
Calcifications are often deposited in the pancreas. This causes pancreatitis. Patients complain of severe abdominal pain in herpes zoster. With pancreatitis of parathyroid origin, the concentration of calcium in the blood usually decreases slightly.
Eyes
Calcium deposits are noted in the vessels of the organ of vision, as well as in the cornea. At an early stage, patients have redness of the eyes. Patients suffer from frequent conjunctivitis.
In the future, tape keratopathy develops. This is a disease in which calcium salts accumulate in the center of the cornea. It is accompanied by pain in the eyes and visual impairment.
Hypercalcemic crisis
Hypercalcemic crisis is a formidable consequence of primary hyperparathyroidism. What it is? This is a life-threatening condition, accompanied by a sharp and rapid increase in the concentration of calcium in the blood. Most often, it appears in the later stages of the disease in the absence of adequate treatment. However, there are cases when a hypercalcemic crisis occurs at an early stage. A complication may develop suddenly amid well-being.
The following factors can trigger a crisis:
- infection;
- pregnancy;
- poisoning;
- fractures of large bones;
- dehydration;
- the use of foods rich in calcium;
- taking diuretics and antacid drugs.
Hypercalcemic crisis always occurs acutely. The patient's well-being is rapidly deteriorating. This dangerous condition is accompanied by the following symptoms:
- unbearable abdominal pain (as with peritonitis);
- fever (up to +39 - +40 degrees);
- incessant vomiting;
- constipation
- bone pain;
- psychomotor agitation;
- coma (in severe cases).
Such a complication leads to death in about half the cases. Severe hypercalcemia causes blood coagulation inside the vessels. Patients die from cardiac arrest or respiratory center paralysis.
What to do with a complication of primary hyperparathyroidism? The clinical recommendations indicate that patients with a hypercalcemic crisis are subject to emergency hospitalization. It is impossible to independently provide assistance at home, so you must immediately call an ambulance team. Such patients are shown emergency surgery on the parathyroid glands. If surgical intervention is not possible, calcium antagonists are administered to patients.
Diagnostics
An endocrinologist is involved in the treatment of this pathology. If surgery is necessary, a surgeon may be required.
Hypercalcemia and osteoporosis are also characteristic of other pathologies. Therefore, it is important to conduct differential diagnosis of primary hyperparathyroidism with the following diseases and conditions:
- bone tumors;
- excess in the body of vitamin D;
- hypercalcemia against the background of other endocrine disorders or diuretics.
Patients are prescribed a blood test for parathyroid hormone. An increased concentration of PTH indicates the presence of hyperparathyroidism.
Then it is necessary to distinguish the primary form of pathology from the secondary. For this purpose, blood and urine tests for calcium and phosphorus are prescribed. In the primary form of the disease, the concentration of calcium is increased in plasma and in urine. In this case, the level of phosphates in the blood is reduced, and in the urine - is increased. If hyperparathyroidism is secondary, then the calcium content in the blood remains within normal limits.
After identifying elevated levels of PTH and hypercalcemia, instrumental diagnosis of primary hyperparathyroidism is performed. This helps establish the etiology of the disease. Patients are prescribed ultrasound, MRI or CT scan of the parathyroid glands. Such examinations reveal the presence of tumors and organ hyperplasia.
Sometimes in patients, an instrumental examination does not show pathological changes in the parathyroid glands. But at the same time, patients have all the signs of primary hyperparathyroidism. The clinical recommendations indicate that in such cases it is necessary to have an MRI of the mediastinum. In this area, additional parathyroid glands can be located, on which adenomas are often formed.
Surgical intervention
This pathology is not subject to drug therapy. Currently, not enough effective drugs have been developed to reduce the production of parathyroid hormone. In addition, adenomas and hyperplasia of the parathyroid glands often progress. Therefore, the most effective way to treat primary hyperthyroidism is surgery.
Indications for surgery are the expressed symptoms of the disease:
- severe osteoporosis;
- plasma calcium concentration of more than 3 mmol / l;
- impaired renal function;
- the formation of stones in the urinary tract;
- excretion of calcium in urine in an amount of more than 10 mmol / day.
If hyperparathyroidism is provoked by an adenoma or malignant tumor, then the doctor excises the neoplasm. With hyperplasia, the surgeon completely removes the three parathyroid glands and part four. Such an operation is called subtotal parathyroidectomy. Currently, surgical operations on the parathyroid glands are often carried out by endoscopic methods.
After surgery, manifestations of primary hyperparathyroidism gradually disappear. The doctor's recommendations during the recovery period after the intervention must be carefully followed. Within 1.5-2 months after removal of the tumor or parathyroidectomy, you need to refrain from heavy physical exertion and sports. In the operated patients, relapses of the disease are noted in 5-7% of cases.
Patient Monitoring
With mild forms of the disease and the absence of indications for surgery, dynamic observation is prescribed. All persons diagnosed with primary hyperparathyroidism are subject to medical records. The register of patients is maintained in the endocrinology clinic. Patients need to regularly see a doctor and undergo the following examinations:
- blood and urine tests for calcium and phosphorus;
- blood pressure measurement;
- Ultrasound of the kidneys;
- blood test for parathyroid hormone level;
- MRI or ultrasound of the parathyroid glands.
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Forecast
With timely treatment of primary hyperparathyroidism, the prognosis of the disease is favorable. After surgery on the parathyroid glands, the patient's well-being is gradually normalizing. Pathological manifestations from the vessels, nervous system and gastrointestinal tract disappear within 1 month after removal of the tumor or parathyroidectomy. The bone structure is fully restored 1-2 years after surgery.
The prognosis is significantly worsened with kidney damage. Such changes are irreversible. Signs of kidney failure persist after surgery.
It can be concluded that hyperparathyroidism is a serious and dangerous disease that disrupts the functions of many organs and systems. It is very important not to miss the first signs of pathology and to start treatment on time.