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 reasons for this violation? And how to recognize the initial signs of the disease? We will answer these questions in the article.
Description of pathology
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 an increased amount, then doctors call this condition hyperparathyroidism. This violation may be due to various reasons. If increased production of parathyroid hormone is caused by pathological changes in the parathyroid glands (tumor orhyperplasia), then experts speak of primary hyperparathyroidism. If this endocrine disorder is provoked by diseases of other organs (most often the kidneys), then it is secondary.
Increased production of parathyroid hormone has an extremely adverse effect on the entire body, and above all on the skeletal system and kidneys. Increased secretion of PTH leads to the removal of calcium from the bones and an increase in its concentration in plasma (hypercalcemia). This causes the following system disturbances:
- formation of fibrous changes in the bones;
- skeletal deformities;
- calcium deposits in the kidneys and on the walls of blood vessels;
- slowing down the transmission of nerve impulses;
- hypertension;
- increased secretion of gastric juice;
- appearance of ulcers in the digestive tract.
In addition, the patient's excretion of phosphorus through the kidneys increases dramatically. This leads to the appearance of stones in the excretory organs.
Primary hyperparathyroidism is much more common in women than in men. Often, symptoms of the disease appear during menopause. This pathology is also called parathyroid osteodystrophy or Engel-Recklinghausen disease. This endocrine disorder is quite common. It is the third most common disorder after diabetes and thyroid dysfunction.
Reasons
The causes of primary hyperparathyroidism are the following pathological changes in the parathyroid glands:
- adenoma;
- hyperplasia;
- malignantswelling.
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 older patients, especially in post-menopausal women.
Much less often, the cause of hyperparathyroidism is the overgrowth of tissue (hyperplasia) of the glands. This pathology is usually hereditary and occurs in young patients. Hyperplasia is often accompanied by dysfunction of other endocrine organs.
Cancer of the parathyroid glands is extremely rare, 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 he althy, but the patient has an elevated PTH hormone. This may indicate a tumor or hyperplasia in additional organs, which makes diagnosis difficult.
Varieties of pathology
As already mentioned, increased production of parathyroid hormone negatively affects the function of various organs. Depending on the location of the lesion and symptoms, doctors distinguish the following forms of primary hyperparathyroidism:
- Bone. With this type of pathology, severe damage to the musculoskeletal system is noted.device. The bones become extremely brittle 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. As a result of hypercalcemia, patients develop kidney and gallbladder stones, and there are signs of atherosclerosis. In addition, ulcers appear in the gastrointestinal tract, vision deteriorates, and the neuropsychic sphere suffers. Pathological changes in the bone tissue are mild.
- Mixed. Patients simultaneously experience damage to bones and internal organs due to hypercalcemia.
ICD: classification of pathology
Primary hyperparathyroidism according to ICD-10 is considered as a violation of the function of the parathyroid glands. This class of diseases is designated by code E21. This group of pathologies includes all endocrine disorders, accompanied by an increase in parathyroid hormone secretion. The full ICD-10 code for primary hyperparathyroidism is E21.0.
Initial symptoms
At an early stage, the disease can proceed without severe symptoms. At the beginning of the pathology, PTH secretion is slightly increased. As a result, a violation of the function of the parathyroid glands is detected late, when the patient has already had a serious lesion of the bones and internal organs. Diagnosis of the disease at an early stage is possible only with the help of a blood test for hormones.
The first signs of an endocrine disorder appear with a significant increase in the secretion of parathyroid hormone. Symptoms and treatment of primaryhyperparathyroidism in women and men depends on the form of the disease. However, it is possible to identify 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 often becomes difficult for patients to get up from a chair without support or enter the door of public transport.
- Musculoskeletal pain. This is the initial sign of leaching of calcium from the tissues. The most common pain is in the feet. Characteristic "duck" gait. Due to the pain syndrome, patients walk, waddling from foot to foot.
- Frequent urination and thirst. In patients with primary hyperparathyroidism, calcium excretion in the urine is increased. 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 loosening and loss of teeth, as well as rapidly progressing caries.
- Slimming, skin color change. The weight of patients in the first months of the disease may decrease by 10-15 kg. Increased diuresis leads to severe dehydration, which causes weight loss. Patients' skin becomes excessively dry and grayish or earthy.
- Neuropsychiatric disorders. Hypercalcemia leads to deterioration of the brain tissue. Patients present with frequent headaches, mood swings,increased anxiety and depression.
Patients do not always associate such symptoms with endocrine disorders. Therefore, a visit to the doctor is often delayed.
At the advanced stage of pathology, the clinic of primary hyperparathyroidism is characterized by a pronounced lesion of bone tissue, blood vessels and internal organs. An increase in the 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 tissue
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 bend easily. There is a curvature of the pelvis, spine, and in severe cases, and limbs. The chest becomes bell-shaped.
- Pain syndrome. Patients suffer from pain in the back and limbs. Often there are attacks that resemble manifestations of gout. This is due to both bone deformities and the deposition of calcium and phosphorus s alts in the joints.
- Frequent fractures. Patients are injured not only with falls and bruises, but even with awkward movements. Sometimes fractures occur spontaneously when the patient is completely at rest. AtIn hyperparathyroidism, traumatization is not always accompanied by severe pain. There are times when patients do not notice fractures. In this case, healing is very slow, as the bones do not grow together well.
- Height reduction. Due to skeletal deformities, the height of patients may be reduced by 10 - 15 cm.
Multiple fractures can lead to disability of the patient. In advanced cases, the patient loses the ability to move independently and serve himself.
Organs of excretion
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 frequent urination and thirst. Over time, stones form in the organ, which is accompanied by bouts of renal colic.
The stronger the signs of kidney damage, the more unfavorable the prognosis of the disease. In severe cases, patients develop swelling and kidney 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 present with symptoms consistent with cardiovascular disease:
- headaches;
- arrhythmia;
- high blood pressure;
- angina attacks.
Calcium deposits canbe formed 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;
- sluggishness;
- headaches;
- dreary mood;
- anxiety;
- drowsy;
- decrease in memory and mental abilities.
In severe cases, patients develop psychotic disorders with clouding of consciousness, delusions and hallucinations.
Gastrointestinal organs
As already mentioned, the hormone PTH affects the secretion of gastric juice. Many patients with hyperparathyroidism have hyperacidity. This is accompanied by the following symptoms:
- abdominal pain of various localization;
- nauseous;
- high gas production;
- 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 s alts can also be deposited in the gallbladder. This leads to inflammation of the organ (cholecystitis), and subsequently to cholelithiasis. There are bouts of pain in the right hypochondrium and nausea.
Calcifications are often deposited in the pancreas. This causes pancreatitis. Patients complain of severe abdominal pain of girdle character. With parathyroid pancreatitisorigin in the blood, the concentration of calcium is usually somewhat reduced.
Eyes
Calcium deposits are noted in the vessels of the organ of vision, as well as in the cornea. At an early stage, patients experience redness of the eyes. Patients suffer from frequent conjunctivitis.
Later on, band keratopathy develops. This is a disease in which calcium s alts accumulate in the center of the cornea. It is accompanied by pain in the eyes and blurred vision.
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 the hypercalcemic crisis occurs at an early stage. The complication may develop suddenly on the background of good he alth.
The following factors can provoke a crisis:
- infectious disease;
- pregnancy;
- poisoning;
- fractures of large bones;
- dehydration;
- eating foods rich in calcium;
- taking diuretics and antacids.
Hypercalcemic crisis always occurs acutely. The patient's condition is rapidly deteriorating. This dangerous condition is accompanied by the following symptoms:
- unbearable abdominal pain (as with peritonitis);
- fever (up to +39 - +40 degrees);
- continuousvomit;
- constipation;
- bone pain;
- psychomotor agitation;
- coma (in severe cases).
This complication is fatal in about half of the cases. Severe hypercalcemia causes blood to clot within the vessels. Patients die from cardiac arrest or paralysis of the respiratory center.
What to do in case of complication of primary hyperparathyroidism? Clinical guidelines indicate that patients with a hypercalcemic crisis are subject to emergency hospitalization. It is impossible to provide assistance at home on your own, so you must immediately call an ambulance team. Such patients are indicated for urgent surgery on the parathyroid glands. If surgery is not possible, patients are given calcium antagonists.
Diagnosis
This pathology is treated by an endocrinologist. If surgery is necessary, a surgeon's consultation may be required.
Hypercalcemia and osteoporosis are also characteristic of other pathologies. Therefore, it is important to make a differential diagnosis of primary hyperparathyroidism with the following diseases and conditions:
- bone tumors;
- excess in the body of vitamin D;
- hypercalcemia due to other endocrine disorders or diuretics.
Patients are prescribed a blood test for parathyroid hormone. Elevated PTH concentration indicates the presence of hyperparathyroidism.
Then you need to distinguishthe primary form of pathology from the secondary. For this purpose, blood and urine tests are prescribed for the content of calcium and phosphorus. In the primary form of the disease, the concentration of calcium is increased in both plasma and urine. At the same time, the level of phosphates in the blood is reduced, and in the urine it is increased. If hyperparathyroidism is secondary, then the calcium content in the blood remains within the normal range.
After detecting an elevated level of PTH and hypercalcemia, an instrumental diagnosis of primary hyperparathyroidism is performed. This helps to establish the etiology of the disease. Patients are prescribed ultrasound, MRI or CT of the parathyroid glands. Such examinations make it possible to detect the presence of tumors and hyperplasia of the organ.
Sometimes in patients, instrumental examination does not show pathological changes in the parathyroid glands. But at the same time, patients have all the signs of primary hyperparathyroidism. Clinical guidelines indicate that in such cases it is necessary to conduct an MRI of the mediastinum. Additional parathyroid glands may be located in this area, on which adenomas often form.
Surgery
This pathology is not subject to drug therapy. Currently, there are no sufficiently effective drugs 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 severe symptoms of the disease:
- heavyosteoporosis;
- plasma calcium concentration over 3 mmol/l;
- kidney disorders;
- stone formation in the urinary tract;
- Calcium excretion in the urine in an amount of more than 10 mmol/day.
If hyperparathyroidism is provoked by an adenoma or a malignant tumor, the doctor will excise the neoplasm. With hyperplasia, the surgeon completely removes three parathyroid glands and part of the fourth. This operation is called a subtotal parathyroidectomy. Currently, surgical interventions on the parathyroid glands are often performed by endoscopic methods.
After the operation, the manifestations of primary hyperparathyroidism gradually disappear. The recommendations of the doctor during the recovery period after the intervention must be carefully followed. Within 1.5-2 months after removal of the tumor or parathyroidectomy, one should refrain from heavy physical exertion and sports activities. In operated patients, relapses of the disease are observed in 5-7% of cases.
Patient monitoring
In mild forms of the disease and there are no indications for surgery, dynamic monitoring is prescribed. All persons diagnosed with primary hyperparathyroidism are subject to medical registration. The register of patients is maintained in the endocrinological dispensary. Patients need to regularly visit a doctor and undergo the following examinations:
- blood and urine tests for calcium and phosphorus;
- measuring blood pressure;
- Ultrasound of the kidneys;
- blood level testparathyroid hormone;
- MRI or ultrasound of the parathyroid glands.
Doctors prescribe a special diet for patients. Foods rich in calcium, primarily milk and dairy products, are excluded from the diet. The patient must drink at least 2 liters of fluid per day. This will help reduce the calcium concentration in the blood and avoid dehydration.
Diet is an important part of the treatment of primary hyperparathyroidism. The recommendations of the doctor regarding the rules of nutrition should be strictly followed. Eating foods high in calcium can trigger a hypercalcemic crisis.
Patients are categorically contraindicated in taking diuretics and cardiac glycosides. These medicines can make the condition worse. If hyperparathyroidism occurs in a woman against the background of menopause, then after consultation with a gynecologist, estrogen replacement therapy may be prescribed.
Forecast
With timely treatment of primary hyperparathyroidism, the prognosis of the disease is favorable. After surgery on the parathyroid glands, the patient's he alth gradually returns to normal. Pathological manifestations from the vessels, nervous system and organs of the gastrointestinal tract disappear within 1 month after removal of the tumor or parathyroidectomy. The bone structure is fully restored in 1-2 years after the operation.
Prognosis worsens significantly with kidney damage. Such changes are irreversible. Signs of kidney failure persist after surgery.
Can doconclusion 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 start treatment on time.
