Clinical picture and treatment of hypoparathyroidism. Trousseau's symptom - the hand of an obstetrician and Khvostek

OBSTETRIC ROTATION, an operation, with the help of a swarm, you can change this position of the fetus, why is it either disadvantageous for the course of labor, to another, more advantageous, moreover, of course, always only to the longitudinal position. (History of A. p. See Obstetric ... ...

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Spasmophilia - SPASMOPHILIA is a disease of young children characterized by a tendency to tonic and clonic-tonic seizures due to hypocalcemia. Etiology and pathogenesis. The content of calcium in blood serum with rickets decreases in ... ... Wikipedia

Tetany - I Tetania (tetania; Greek tetanos tension, convulsion) is a pathological condition characterized by convulsive syndrome and increased neuromuscular excitability due to a decrease in the concentration of ionized calcium in the blood serum, as ... ... Medical encyclopedia

Smellie-Siegemundin reception - (W. Smellie, 1697 1763, English obstetrician; J. Siegemundin, 1648 1705, German midwife; synonym double manual reception) obstetric rotation of the fetus on the leg, in which one hand of the obstetrician, inserted into the uterus, pushes the head up, and the other pulls the leg by ... ... Big Medical Dictionary

Smellie - Siegemund's reception - (W. Smellie, 1697 1763, English obstetrician; J. Siegemundin, 1648 1705, German midwife; synonym double manual reception) obstetric rotation of the fetus on the leg, in which one hand of the obstetrician, inserted into the uterus, pushes the head up, and the other pulls ... ... Medical encyclopedia

Spasmophilia - I Spasmophilia (spasmophilia; Greek spasmos spasm, spasm + philia inclination; synonym: rickytogenic tetany, spasmophilic diathesis) is a pathological condition that occurs in children with rickets in the first 6-18 months. life; characterized by ... ... Medical encyclopedia

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Rigid person syndrome - Syn .: Syndrome of "stiff man". Mersh-Woltman syndrome. Progressive symmetrical muscle stiffness, usually manifested in men aged 20-40 years. Muscle tension and painful muscle spasms build up over several ... ...

Bullet Phenomenon - spasm of the hand muscles ("obstetrician's hand") during passive extension of the arm (with spasmophilia, tetany) ... Encyclopedic Dictionary of Psychology and Pedagogy

Hypoparathyroidism (tetany) is a disease caused by decreased production of parathyroid hormone and characterized by attacks of tonic seizures and hypocalcemia. The disease was first described by Kussmaul in 1872.

Etiology and pathogenesis
The etiological factors causing the development of hypocalcemia can be divided into 4 groups:

Diseases and conditions leading to insufficient production of parathyroid hormone: autoimmune processes in the parathyroid glands, removal of the parathyroid glands during strumectomy, destruction of the parathyroid glands during treatment with radioactive iodine; trauma, sarcoidosis of the parathyroid glands; Di Georges syndrome (agnesia of the parathyroid glands, aplasia of the thymus gland, congenital malformations, immunological insufficiency); infectious diseases (tuberculosis, flu, rheumatism, etc.), physical or nervous overstrain, hypothermia and overheating. In rare cases, tetany has a neurogenic origin;

Functional hypoparathyroidism can develop in the following cases: in newborns born to mothers suffering from hyperparathyroidism; with idiopathic neonatal hypocalcemia; with hypomagnesemia (malabsorption, vomiting, diarrhea, steatorrhea, diabetes mellitus, alcoholism); with a lack of vitamin D;

Peripheral resistance to parathyroid hormone can be caused by pseudohypoparathyroidism (Albright's syndrome), chronic renal failure, vitamin D deficiency;

Hypocalcemia of an iatrogenic nature is associated with the administration of certain drugs to the patient: phosphates, mitromycin, neomycin, actinomycin, thiazide diuretics.
In addition, hypocalcemia can be triggered by prolonged use of laxatives, massive infusion of citrated plasma, and overdose of calcitonin.

A decrease in parathyroid hormone production leads to disruption of various forms of metabolism, in particular, to poor absorption of calcium. In addition, insufficient production of the parathyroid hormone causes an increase in the level of phosphorus in the blood (hyperphosphatemia).

A decrease in the concentration of calcium in the blood serum causes an increase in nervous and muscle excitability with the subsequent development of the tetany symptom complex, manifested by convulsive contractions of striated and smooth muscles.

Clinical picture
The clinical symptoms of hypoparathyroidism are due to calcium deficiency. The main symptom of the disease is attacks of tonic seizures, covering many muscle groups. The attack occurs spontaneously or is provoked by mechanical or acoustic influences or hyperventilation. An attack of tetany begins suddenly or with precursors (general weakness, crawling creeps, numbness or tingling of the extremities, paresthesia in the face). Next comes a painful tonic contraction of the muscles of the upper and lower extremities, muscles of the face, diaphragm (can be triggered by respiratory failure), stomach, intestines (abdominal pain). Bronchospasm and laryngospasm with stridor often occur, which are especially dangerous for children. Muscle cramps of the upper extremities are characterized by a predominance of flexor muscle tone, as a result of which the hand looks like an "obstetrician's hand".

With cramps of the lower extremities, the tone of the extensor muscles predominates, therefore plantar flexion occurs - "horse foot".

In connection with the tonic contractions of the mimic muscles, the patient's mouth has a peculiar appearance - "fish mouth". Spasms of the muscles of the facial muscles are accompanied by trismus, cramps of the eyelids, a characteristic "sardonic smile". Muscle cramps are very painful. Spasms of smooth muscles of the ureters and bile ducts are manifested in the form of renal or hepatic colic. Spasm of the sphincter of the bladder is accompanied by urinary retention. There are attacks of migraine, angina pectoris, Raynaud's syndrome, associated with spasm of the vascular muscles. The patient's consciousness during an attack of tetany is preserved. The skin is pale, damp, and cold to the touch. The puffiness of the face is noted. Trophic disorders lead to brittle nails, hair loss, caries. The patient's skin is often affected by dermatosis and eczema.

Laboratory tests reveal lymphocytosis, eosinophilia, hyperglobulinemia, hyperphosphatemia, and often proteinuria. A characteristic symptom of tetany is hypocalcemia (below 2 mmol / l - 8 mg%).

A positive test of Khvostek is a diagnostic sign of the disease - when tapping the facial nerve at the tragus of the auricle, the muscles of the corresponding half of the face contract. Trousseau's symptom is characteristic of tetany: forcing air into the tonometer's cuff, located on the shoulder, above the maximum blood pressure figures, causes a contraction of the hand muscles ("obstetrician's hand"). In addition, patients with tetania have positive symptoms of Ashner (pressure on the eyeballs causes a sharp slowdown in the pulse) and Schlesinger (when the leg is bent in the hip joint, the foot acquires the position of a “horse foot.” When the ulnar nerve is irritated by galvanic current (0.8–1 mA ) in patients with hypoparathyroidism, muscle contraction and tetanus occur.

The course of the disease depends on the etiological factor causing its occurrence. The disease takes on a severe, violent course during postoperative or traumatic tetany. The postoperative form of the disease can quickly result in death. In other forms of tetany, the course and prognosis are relatively favorable.

Treatment
To stop an attack of tetany, the patient is injected intravenously with a 10% solution of calcium chloride (10–20 ml) or a solution of calcium gluconate (the dose is 2 times greater than that of calcium chloride). With these drugs, the seizure usually stops quickly.

A slower effect (after 2-3 hours) has the introduction of parathyroid hormone. It is prescribed in a dose of 2-4 ml subcutaneously or intramuscularly every 3-4 hours until the attack stops completely. Outside of an attack, parathyroid hormone is prescribed 1–2 ml daily or every other day under the control of the level of calcium in the blood. With prolonged treatment with this drug, resistance to parathyroid hormone develops due to the appearance of antibodies to it. Therefore, it is currently not recommended to resort to long-term parathyroid hormone replacement therapy.

With neurogenic tetany, tranquilizers are prescribed (seduxen, elenium, etc.). After stopping an attack of hypocalcemia, therapy with calcium preparations (calcium chloride, calcium gluconate, etc. - up to 10 g per day) and vitamin D (ergocalciferol, dihydrotachysterol, videchol, etc.) and a diet low in phosphorus and high in calcium is recommended.

Spasmophilia (tetany) is a painful condition in which there is increased neuromuscular excitability with a tendency to convulsions and spastic manifestations, usually detected in early spring during a period with increased insolation. Occasionally in adults, spasmophilia affects mainly children aged from 6 months to 3 years, premature babies and who are on artificial and mixed feeding. Spasmophilia occurs when there is a calcium deficiency in the child's body and is often combined with rickets.

Causes of spasmophilia in children

Spasmophilia develops as a result of a lack of calcium in the blood due to the intake of high doses of vitamin D in the treatment of rickets or with an increase in insolation and excessive production of this vitamin in the skin. Its overproduction in the body has a negative effect on the function of the thyroid gland, provokes the absorption of calcium and phosphorus salts in the intestine and their further reabsorption (reabsorption) in the renal tubules, which is the cause of alkalosis. Active deposition of calcium in the bones leads to a decrease in its amount in the blood - hypocalcemia, and also increases neuromuscular excitability, leading to seizures.

Symptoms of latent spasmophilia

The disease can have a latent (latent) or explicit form. The latent form of spasmophilia in children is very difficult to determine - they are actually healthy, they have normal physical and psychomotor development, but at the same time, symptoms of rickets often appear. There are a number of symptoms characteristic of spasmophilia that confirm its latent form. Let's name some of them:

  • Khvostek's symptom. It is characterized by contraction of the facial muscles of the face with light tapping on the cheek between the corner of the mouth and the earlobe along the trunk of the facial nerve;
  • Weiss symptom. Characterized by the occurrence of contraction of the muscles of the forehead and eyelids when tapping with a percussion hammer in the region of the edge of the orbit from the outside;
  • Trousseau's symptom. With an intense tug of the shoulder with a tourniquet or cuff, a convulsive contraction of the hand muscles occurs in the outstretched arm. This results in an obstetrician hand position. Convulsions with spasmophilia are preceded by a feeling of numbness and soreness in the fingers;
  • Maslov's symptom. In children with spasmophilia, in contrast to healthy children, when a needle is injected into the leg, not only rapid breathing is observed, but also apnea (short-term respiratory arrest) recorded by a pneumograph.

The latent form of spasmophilia in children is determined quite often. Under the influence of certain factors, such as fright, vomiting, sudden insolation, an increase in temperature with an infectious disease, it can turn into an explicit form. If spasmophilia is detected, treatment should be carried out immediately.

The clinical picture and symptoms of overt spasmophilia

An explicit form of spasmophilia in children can be expressed by laryngospasm, carpopedic spasm, eclampsia, or a combination thereof:

  • Laryngospasm. One of the most common forms of spasmophilia, also called the "parent," is characterized by a sudden acute narrowing of the glottis, with its possible partial or complete closure, which occurs when crying or fright. With a moderate manifestation of laryngospasm, the child's skin turns pale, cold sweat appears, and the voice changes with a hoarse or sonorous breath. The attack can last up to 2 minutes, then when breathing is restored, the child falls asleep. In the absence of adequate treatment for spasmophilia, attacks can be repeated, during a pronounced laryngospasm, sometimes there is a complete cessation of breathing and loss of consciousness, in the most severe cases, a lethal outcome is possible;
  • Carpopedic spasm. Symptoms of spasmophilia in this form of the disease are tonic cramps of the hands, feet and face. At the same time, the child has arms bent at the elbows, hands lowered down with a characteristic Trousseau symptom, bent legs and feet. Attacks of spasmophilia can last from several minutes to several hours, while reactive edema of the hand and foot appears, spasms of the respiratory muscles are also possible, carrying the threat of holding and stopping breathing and spasms of the heart muscle with possible cardiac arrest;
  • Eclampsia. The rarest and most dangerous form of spasmophilia, manifested by attacks of clonic-tonic seizures, spreading to the entire muscles. With a mild course, cramps of the muscles of the face are characteristic, the appearance of cyanosis, intermittent breathing and numbness of the child. During a severe course of an attack of spasmophilia, convulsions of the whole body, loss of consciousness, biting of the tongue, involuntary urination and defecation occur, the attack can last up to 25 minutes. In severe cases, respiratory and cardiac arrest is possible, most often eclampsia affects children in the first year of life.

These forms of spasmophilia pose a threat to the health and life of children and require emergency medical attention to carry out urgent resuscitation actions.

Spasmophilia in adults

Spasmophilia in adults is much less common than in children, it can be caused by the following reasons:

  • Surgery to remove the parathyroid glands;
  • Hemorrhage;
  • Tumors that have a negative effect on the thyroid gland.

In some cases of spasmophilia in adults, its etiology remains unknown. The factors provoking the detection of the disease in a latent form include pregnancy, lactation, infectious diseases, and nervous tension. To prevent attacks of spasmophilia, it is recommended to avoid stressful situations, be able to control breathing and relax, and include foods containing calcium, magnesium and phosphorus in the diet.

Spasmophilia treatment

When diagnosing spasmophilia in children, the age of the child, the season, the presence of signs of rickets are taken into account, laboratory tests determine the symptoms of hypocalcemia, hypophosphatemia, metabolic alkalosis.

In the treatment of spasmophilia, the relief of convulsive syndrome is carried out with the help of anticonvulsants - seduxen, relanium, magnesium sulfate, gamma-aminobutyric acid. To restore the level of calcium in the blood and eliminate alkalosis, a 10% solution of calcium gluconate and 3-5% ammonium chloride is used. In the future, vitamin therapy is carried out, and after normalization of the level of calcium in the blood, therapeutic doses of vitamin D are prescribed.

When an attack of laryngospasm develops, before the arrival of an ambulance, it is necessary to lay the patient on a hard surface, ensure peace and access to air, sprinkle the face and body with cold water, ammonia or other means to irritate the nasopharyngeal mucosa. To stop an attack of spasmophilia, physicians inject a solution of calcium and magnesium intravenously, and intramuscularly sedatives. If the measures taken are ineffective and there is no breathing, tracheal intubation is necessary, as well as indirect heart massage when it stops.

Prevention of spasmophilia consists in the timely diagnosis and adequate treatment of rickets.

Acute hypoparathyroidism - an acute pathological condition that develops as a result of insufficient increment of parathyroid glands of parathyroid hormone, which leads to a sharp decrease in the level of calcium in the blood, followed by attacks of tonic seizures.

For the first time tetany was described by J. Clarke (1815). Tetania occurs at any age. It occurs not only with hypoparathyroidism, but also with a number of diseases and conditions without a decrease in the function of the parathyroid glands. The term "tetany" in contrast to "acute hypoparathyroidism" is a collective concept.

Etiology and pathogenesis of acute hypoparathyroidism

Acute hypoparathyroidism most often occurs due to accidental removal or trauma of the parathyroid glands during thyroid surgery. The frequency of tetany after surgery on the thyroid gland is in the range from 0.4 to 3.1% [Gurevich GM, Mastbaum IS, 1968]. According to other data, the frequency of tetany during resection of the thyroid gland is much higher and reaches 10% [Cherenko MP, 1977] and even up to 20.5%.

A hypocalcemic crisis can develop when a hyperfunctioning parathyroid adenoma is removed as a result of atrophy of other parathyroid glands. The frequency of tetany after parathyroid adenomectomy is 50-67% [Nikolaev OV, Tarkaeva VN, 1974]. In some cases, tetany that appears after the removal of parathyroid adenoma is functional and disappears with compensatory hyperplasia of the remaining parathyroid glands. Tetany can result from postoperative inflammation of the parathyroid glands or their autoimmune damage, as well as from damage to the parathyroid glands by metastases of malignant neoplasms. Etiological factors of tetany can also be tuberculosis, measles, influenza [Yules M., Hollo I., 1967], amyloidosis of the parathyroid glands, radioactive iodine treatment of toxic goiter. In some cases, tetany is a consequence of idiopathic hypoparathyroidism, the cause of which has not been finally established.

The cause of tetany may be congenital inferiority of the parathyroid glands. With latent parathyroid gland insufficiency, it can be detected as a result of infection, intoxication (poisoning with lead, carbon monoxide, ergot), D-hypovitaminosis, alkalosis, pregnancy, lactation, with insufficient absorption of calcium in the intestine.

Due to the deficiency of parathyroid hormone, the flow of calcium from the bone tissue into the blood decreases and the reabsorption of phosphorus in the proximal renal tubules increases. This leads to hypocalcemia and hyperphosphatemia. A decrease in the content of calcium in the blood leads to an imbalance between sodium and potassium ions, on the one hand, and calcium and magnesium, on the other. As a result, neuromuscular excitability sharply increases.

Tetania develops if the calcium content in the blood decreases to 1.25-1.75 mmol / l (5-7 mg%).

Clinic of acute hypoparathyroidism

Often, an attack of tetany is preceded by precursors - cold extremities, numbness, tingling and creeping, paresthesias and a feeling of spasms. Following the precursors, painful tonic convulsions develop, which more often affect symmetrical muscle groups and are selective.

Most often, cramps occur in the muscles of the upper limbs, less often in the lower ones. Flexor muscles are mainly affected. As a result of spasm of the facial muscles, a sardonic smile appears, the lips take on the appearance of a "fish mouth". With a spasm of the masticatory muscles, convulsive contraction of the jaws (trismus) occurs. Cramps in the muscles of the upper extremities lead to the characteristic position of the hand: fingers are clenched and slightly brought to the palm, the first finger is brought together, the hand is bent at the wrist joint ("obstetrician's hand"). With a spasm of the muscles of the lower extremities, the thighs and lower legs are stretched, the feet are turned inward, the torso bends backward (opisthotonus). Due to convulsive contractions of the intercostal muscles, abdominal muscles and diaphragm, breathing is sharply disturbed.

In children, spasm of the muscles of the larynx is often observed, which leads to laryngospasm. With prolonged laryngospasm, asphyxia occurs and death occurs.

Changes in organs and systems during tetany depend on the predominance of the tone of the sympathetic or parasympathetic system. The predominance of the tone of the sympathetic system leads to tachycardia, increased blood pressure, pallor due to spasm of peripheral vessels, and parasympathetic - to vomiting, bradycardia, decreased blood pressure, diarrhea, and polyuria. Spasms of smooth muscles of internal organs and blood vessels can simulate cardiovascular diseases (attacks of angina pectoris, endarteritis, migraines, etc.), respiratory diseases (bronchial asthma, etc.), gastrointestinal tract (cholecystitis, pancreatitis, appendicitis , stomach ulcer, etc.), genitourinary system (cystitis, nephritis, etc.). On the ECG, an extension of the Q-T interval is noted, which is associated with hypocalcemia.

Attacks of squeezing pains behind the sternum and in the region of the heart such as angina pectoris are distinguished as a kind of pseudoanginous form of tetany [Kyrge K. X., 1956; Quandt J., 1954, et al.]. The cardiac form of tetany can result in death as a result of a convulsive contraction of the heart muscle or spasm of the coronary vessels. In some cases, spasm of the spinal vessels occurs, as a result of which toxic-vascular myelopathy develops (girdle pain in the thoracic spine, feeling of numbness in the legs, increasing weakness in them to the degree of paraparesis, etc.) [Martynov Yu. S. et al. , 1973].

Spasms of the digestive system, proceeding under the guise of cholecystitis, appendicitis, pancreatitis, etc., in some cases can lead to unjustified surgical intervention. A seizure attack can last from several minutes to several hours. Consciousness during a hypocalcemic crisis is usually preserved. In severe attacks of tetany, loss of consciousness is noted, which, in combination with convulsions, makes them similar to epileptic seizures. On the EEG in these patients, as in epilepsy, peak-wave complexes (peaks, sharp waves, isolated or in combination with a subsequent slow wave) are noted. Under the influence of treatment, these changes can completely or partially disappear. Sometimes patients with tetany develop acute psychosis, cerebral edema, stem and extrapyramidal symptoms.

During an attack of tetany, diplopia may occur due to temporary strabismus or a violation of convergence.

With a mild form of hypoparathyroidism, tetany attacks are rarely observed (1-2 times a week), their duration does not exceed several minutes. In a severe form of hypoparathyroidism, attacks are frequent (sometimes several times a day), last several hours and easily arise under the influence of external stimuli (mental and physical trauma, hypothermia, overheating, etc.). The acute form of hypoparathyroidism has a severe course. Attacks of tonic seizures are often repeated and are protracted.

Laboratory data. For the express diagnosis of hypocalcemic crisis, the determination of the level of calcium in the blood is used. There is a decrease in the level of total calcium to 1.9 mmol / l (7.5 mg%) and below, and ionized - to 1.1 mmol / l (4.3 mg%) and below. The calcium content in the blood usually corresponds to the severity of tetany. In severe form, its calcium content in the blood decreases to 1.5-1.2 mmol / l (6-5 mg%) and below. Hyperphosphatemia is characteristic. Hypocalciuria and hypophosphaturia are noted.

For an approximate judgment about the content of calcium in the blood, the Sulkovich test is used (see the section "Acute hyperparathyroidism"). In urine, calcium is not detected when its content in the blood is below 2-1.75 mmol / l (8-7 mg%).

To diagnose a hypocalcemic crisis with a predominance of symptoms of autonomic dysfunction and viscero-vascular pathology and latent tetany, tests based on increased mechanical are used. thermal and electrical excitability of the neuromuscular apparatus.

When tapping with a finger or a percussion hammer along the facial nerve in front of the tragus of the auricle (at the exit site of the facial nerve), Khvostek's symptom is revealed. This symptom can be grade I, II, or III. Muscle contraction of the entire area innervated by the facial nerve is characteristic of overt tetany and is referred to as "Tail-I". With "Khvostek-P" there is a contraction of the muscles in the area of \u200b\u200bthe wing of the nose and the corner of the mouth, and with "Khvostek-III" only the muscles of the corner of the mouth contract. "Khvostek-P" and "Khvostek-Sh" are usually found with latent tetany.

Trousseau's symptom is detected by pulling the patient's shoulder with a rubber band or rubber cuff until the pulse disappears for 2-3 minutes. With latent tetany, after a specified time, a convulsive reaction of the hand in the form of an "obstetrician's hand" occurs in the stretched limb.

Schlesinger's symptom is found with rapid passive flexion in the hip joint of the patient's leg, straightened in the knee joint. With latent tetany, a convulsive contraction of the extensor muscles of the thigh appears with a sharp supination of the foot. The contraction of the muscles of the extremities under the influence of a galvanic current of even small strength (0.7 mA) is called the Erb symptom.

Diagnostics and differential diagnosis of acute hypoparathyroidism

The diagnosis of acute hypoparathyroidism is established on the basis of anamnesis (the occurrence of tetany attacks after surgery on the thyroid gland, removal of parathyroid adenoma, radioactive iodine treatment of toxic goiter, etc.), characteristic clinical symptoms (local painful tonic convulsions affecting symmetrical muscle groups - trismus, opistotonus etc., difficulty breathing, convulsive convulsions of the whole body, etc.), laboratory data (hypocalcemia, hyperphosphatemia) and diagnostic tests (symptoms of Khvostek, Trusso, Erb). Symptoms that indirectly indicate chronic hypoparathyroidism are of some importance in the diagnosis of acute hypoparathyroidism: dry skin, brittleness and striation of nails, defects in tooth enamel, caries, central or subcapsular cataracts, alopecia or total baldness, early graying of hair, symmetric calcification and vascular plexus other vessels of the brain, calcification of the basal ganglia, etc.

Seizures in acute hypoparathyroidism are primarily differentiated from other hypocalcemic tetany not associated with the primary pathology of the parathyroid glands. Tetania in these cases may result from impaired absorption of calcium in the intestine due to vitamin D deficiency (spasmophilia, rickets in adults). The cause of tetany can also be various diseases and conditions accompanied by impaired absorption of calcium in the intestine due to steatorrhea (sprue, chronic pancreatitis, diarrhea after resection of a large section of the small intestine, chronic enteritis, etc.). In some cases, such tetany develops due to an increased need for calcium and vitamin D during pregnancy and lactation. Hypocalcemic tetany can also occur after oxalate poisoning.

In contrast to tetany in acute hypoparathyroidism, the above tetany is characterized not only by hypocalcemia, but also by hypophosphatemia. The only exception is tetany after oxalate poisoning, which is characterized by hypocalcemia and normal phosphorus levels in the blood. The cause of hypocalcemia in this case is calcium inactivation due to the formation of insoluble calcium oxalate compounds.

Hypocalcemic tetany, but with hyperphosphatemia, is observed with a congenital disease - Albright's disease, which is called pseudohypoparathyroidism due to the insensitivity of the renal tubules to parathyroid hormone. Patients with pseudohypoparathyroidism are characterized by short stature, short bones, often severe mental retardation and a tendency of subcutaneous tissue to calcify and ossify.

Hypocalcemia with hyperphosphatemia, but usually without tetany, occurs in chronic renal failure. The absence of tetany in this form of hypocalcemia is due to acidosis due to hyperazotemia and hypermagnesemia, however, when the acid-base state shifts to the alkaline side, calcium ionization decreases and tetany may occur. Sometimes hypocalcemic tetany develops due to a magnesium deficiency in the body. The latter helps to suppress the mobilization of calcium from bone tissue by parathyroid hormone. Hypo-magnesium can be in severe nutritional disorders due to malabsorption in the intestine, after prolonged parenteral nutrition, in chronic alcoholism, etc.

Tetany with normal levels of calcium and phosphorus in the blood is observed in alkalosis (gastric and hyperventilating tetany) and citrate poisoning. Gastric tetany occurs after prolonged vomiting and loss of hydrochloric acid, as well as after taking large amounts of sodium bicarbonate, as a result of which the concentration of ionized calcium in the blood decreases. However, the total blood calcium level remains within the normal range. Hyperventilation tetany is caused by the increased release of CO 2, as a result of which alkalosis occurs. Hyperventilation tetany (after frequent and deep respiratory movements) is observed in hysteria and less often in patients with encephalitis.

Tetany in citrate poisoning is due to calcium inactivation due to the formation of calcium citrate.

M. Jules and I. Hollo (1967) proposed the following scheme for the differential diagnosis of tetany not associated with the primary pathology of the parathyroid glands (Scheme 1).

Tetania can be of iatrogenic origin (administration of large doses of calcitonin, mithramycin, EDTA, glucagon, phosphorus salts, prolonged use of phenobarbital, in connection with which the metabolism of vitamin D is disturbed, etc.).

Seizures in acute hypoparathyroidism of diffetania caused by organic diseases of the central nervous system (rheumatism, brain tumor, etc.), primary hyperaldosteronism, tetanus, rabies, strychnine poisoning.

In contrast to a hypocalcemic crisis in epilepsy, the symptoms of Khvostek, Trousseau, Erba are usually negative and phosphorus-calcium metabolism is not disturbed. The hypoglycemic genesis of seizures is evidenced by anamnesis, characteristic hunger, normal blood calcium in the presence of hypoglycemia.

In tetany syndrome caused by organic diseases of the central nervous system, in contrast to tetany in acute hypoparathyroidism, there is no violation of phosphorus-calcium metabolism and changes in acid-base balance.

The characteristic symptoms of the disease speak of primary hyperaldosteronism: attacks of severe muscle weakness, transient flaccid paralysis, arterial hypertension, polyuria, polydipsia, hypokalemia, etc. The calcium content in the blood is within normal limits.

Tetanus and hypocalcemic crisis can be talked about based on the history (the presence of trauma and contamination of the wound with soil) and the widespread nature of seizures, usually starting with the chewing muscles and seizing other muscles in a certain sequence (facial muscles, muscles of the neck, trunk, lower extremities). Unlike a hypocalcemic crisis with tetanus, the calcium content in the blood is within normal limits.

Anamnesis (bite, salivation of an infected animal) and a characteristic clinical picture (hydrophobia, aerophobia, agitation, sometimes violent attacks, alternating with paralysis of the muscles of the extremities, tongue, increased salivation, etc.) indicate the presence of rabies and the absence of hypocalcemic crisis.

In case of strychnine poisoning, in contrast to the hypocalcemic crisis, the symptoms of Chvostek and Trusso are negative, the calcium content in the blood is normal.

Prognosis of acute hypoparathyroidism

With timely and correct replacement therapy, the prognosis for life is usually favorable. With laryngospasm and cardiac tetany, the prognosis is serious (death can occur due to asphyxia, convulsive contraction of the heart muscle, or spasm of the coronary vessels).

Prevention. Prevention of acute hypoparathyroidism consists in maximizing the protection of the parathyroid glands during operations on the thyroid gland, as well as in preventing complications (adhesions, infiltrates, etc.) that disrupt their blood supply after surgery.

Early detection of neuromuscular excitability in patients after thyroid surgery, followed by immediate rational specific therapy, is necessary. Preventive measures should be aimed at preventing infections and intoxications that damage the parathyroid glands, as well as factors provoking the development of an acute hypocalcemic crisis in patients with chronic hypoparathyroidism. In order to prevent tetany, patients with hypoparathyroidism are prescribed a diet rich in calcium and poor in phosphorus.

Treatment of acute hypoparathyroidism

1. For relief of an acute attack of tetany, depending on the severity of the attack, 10-50 ml of a 10% solution of calcium chloride or calcium gluconate is injected intravenously. The effect of the drug appears already at the end of the injection. If necessary, intravenous administration of calcium preparations is repeated 3-4 times a day.

2. After the introduction of calcium preparations to prevent attacks of tetany, 40-100 U of parathyroidin is injected subcutaneously or intramuscularly. Parathyroidin - an extract of the parathyroid glands of cattle - contains 20 IU of the active principle in 1 ml. The therapeutic effect after its administration occurs after 2-3 hours and lasts 20-24 hours. The maximum increase in the level of calcium in the blood after the administration of the drug occurs after 18 hours.

3. After elimination of seizures, dihydrotachysterol (AT-10) is prescribed instead of parathyroidin or simultaneously with it at a dose of 2 mg orally after 6 hours. Further, every 2 days, the dose is reduced by 2 mg. The maintenance dose is usually 2 mg. Dihydrotachysterol not only promotes the absorption of calcium by the intestines, but, like parathyroid hormone, promotes the mobilization of calcium from the bones and the excretion of phosphorus in the urine. Treatment with dihydrotachysterol, like parathyroidin, is carried out under the systematic control of the level of calcium in the blood and its excretion in the urine. The duration of action of the drug after its withdrawal is 10 days. For better intestinal absorption of calcium, ergocal-ciferol (vitamin D2) can be used instead of dihydrotachysterol. To eliminate attacks of tetany, vitamin D2 (alcohol solution) is prescribed at 200,000-400,000 ME per day, followed by a gradual decrease in the dose after the cessation of attacks to 25,000-50,000 ME per day. Treatment with vitamin D2 is carried out under systematic control (1-2 times a month) of the level of calcium in the blood.

4. Prescribe sedatives and antispasmodics (bromides, luminal, chloral hydrate inside or in an enema, papaverine, etc.).

5. To eliminate alkalosis, ammonium chloride is used at 3-7 g per day.

6. With increasing laryngospasm, intubation or tracheotomy is indicated.

V. V. Potemkin Emergencies in the clinic of endocrine diseases, 1984

- a disease of young children, characterized by a violation of mineral metabolism, increased neuromuscular excitability, a tendency to spastic and convulsive states. Latent spasmophilia is manifested by a contraction of the muscles of the face, hand ("obstetrician hand"), short-term cessation of breathing in response to provocation; overt spasmophilia occurs in the form of laryngospasm, carpopedal spasm and eclampsia. The diagnosis of spasmophilia is confirmed by the presence of hypocalcemia, hyperphosphatemia, and a connection with rickets. Treatment of spasmophilia includes help with general convulsions and laryngospasm, prescription of calcium supplements, and adequate therapy for rickets.

General information

Spasmophilia (children's tetany) is a calciopenic condition accompanied by a spastic state of the muscles of the face, larynx, limbs, general clonic or tonic convulsions. Spasmophilia is detected mainly at the age of 3 months to 2 years, that is, in the same age range as rickets, with which child tetany has an etiological and pathogenetic relationship. A few years ago, spasmophilia in pediatrics was quite common, but a decrease in the incidence of severe forms of rickets has led to a decrease in the number of cases of childhood tetany. Spasmophilia develops in 3.5 - 4% of children with rickets; somewhat more often in boys.

Clinical manifestations of spasmophilia in the form of laryngospasm or general seizures with loss of consciousness and respiratory arrest require from any medical specialist and parents the ability and willingness to provide emergency care to the child. A prolonged tetanoid state can cause damage to the central nervous system, a subsequent delay in the child's mental development, and in severe cases, death.

Causes of spasmophilia

Spasmophilia is a specific disorder of calcium-phosphorus metabolism that accompanies the course of moderate to severe rickets. The pathogenesis of spasmophilia is characterized by a decrease in the concentration of total and ionized calcium in the blood against the background of hyperphosphatemia and alkalosis. An increase in the level of inorganic phosphorus in the blood can be facilitated by feeding a child with cow's milk with a high concentration of phosphorus and insufficient excretion of excess phosphorus by the kidneys; hypoparathyroidism. In addition to calcium-phosphorus metabolism, with spasmophilia, hyponatremia, hypochloremia, hypomagnesemia and hyperkalemia are noted.

The clinic of spasmophilia usually develops in early spring with the onset of sunny weather. Intense UV exposure to the child's skin causes a sharp increase in the blood concentration of 25-hydroxycholecalciferol, an active metabolite of vitamin D, which is accompanied by suppression of the function of the parathyroid glands, a change in blood pH, hyperphosphatemia, increased calcium deposition in bones and a critical drop in its blood level (below 1 , 7 mmol / L).

Hypocalcemia causes increased neuromuscular excitability and convulsive readiness. In these conditions, any irritants (fear, crying, strong emotions, vomiting, high fever, intercurrent infections, etc.) can provoke a convulsive attack in a child.

It is noticed that children who are breastfed almost never develop spasmophilia. The risk group includes premature babies, artificially fed children living in unfavorable sanitary and hygienic conditions.

Classification of spasmophilia

Depending on the clinical course, latent (latent) and manifest (explicit) forms of spasmophilia are distinguished, while the transition of latent tetany to explicit one is not excluded.

Latent spasmophilia is detected exclusively with a special examination using mechanical and galvanic skin tests. In this case, characteristic symptoms arise (Khvostek, Erba, Trousseau, Lusta, Maslova).

Manifest spasmophilia can occur in the form of laryngospasm, carpopedal spasm and an attack of eclampsia.

Symptoms of spasmophilia

Latent form

The course of latent spasmophilia always precedes an overt one and can last for several weeks or months. Children with a latent form of spasmophilia usually have signs of rickets: increased sweating, tachycardia, poor sleep, emotional lability, increased anxiety, fearfulness, and digestive disorders. The following symptoms indicate the presence of a latent form of spasmophilia:

  • khvostek symptom (upper and lower) - twitching of the corner of the eye or mouth when tapping with a hammer or finger along the zygomatic arch or the corner of the lower jaw (i.e., the places where the branches of the facial nerve exit);
  • trousseau symptom - compression of the brachial neurovascular bundle leads to convulsive contraction of the hand muscles, bringing it to the position of the "obstetrician's hand";
  • lust's symptom - tapping in the area of \u200b\u200bthe head of the fibula, in the projection of the exit of the peroneal nerve, accompanied by plantar flexion and abduction of the foot to the side;
  • erb's symptom - in case of irritation by galvanic current by force
  • maslov phenomenon - painful irritation (injection) causes a short-term cessation of breathing in a child suffering from latent spasmophilia (there is no such reaction in healthy children).

Manifest spasmophilia

Laryngospasm, as a manifestation of overt spasmophilia, is characterized by a sudden spasm of the muscles of the larynx, which often develops when the child is crying or for no apparent reason. A moderate attack of laryngospasm is accompanied by partial blockage of the airways, pallor, wheezing, reminiscent of a whooping cough reprise. In severe cases, occurring with complete closure of the glottis, cyanosis, cold sweat, short-term loss of consciousness, apnea for several seconds occurs, followed by a sonorous exhalation and noisy breathing; the child calms down and falls asleep. Spasm of the muscles of the larynx with spasmophilia can recur several times during the day. In extreme cases, an attack of laryngospasm can lead to the death of the child.

Another manifestation of obvious spasmophilia is carpopedic spasm - tonic spasm of the muscles of the hands and feet, which can last for hours or days. At the same time, there is maximum flexion of the arms in large joints, bringing the shoulders to the body, flexion of the hands (fingers are gathered into a fist or bent like an "obstetrician's hand" - fingers I, IV, V are bent; II and III - unbent); plantar flexion of the foot, fingers tucked in. Prolonged carpopedic spasm with spasmophilia can lead to reactive edema of the dorsum of the hands and feet.

With spasmophilia, isolated spasms of the eye muscles (transient squint), masticatory muscles (trismus, stiffness of the occiput), smooth muscles (impaired urination and defecation) can also occur. The most dangerous are spasms of the respiratory muscles, leading to dyspnea, bronchospasm and respiratory arrest, as well as the heart muscle, which threatens cardiac arrest.

The most severe form of manifest spasmophilia is eclampsia or a general attack of clonic-tonic seizures. The development of an attack of eclampsia is preceded by twitching of the facial muscles, then the cramps spread to the limbs and muscles of the trunk. General convulsions are accompanied by laryngospasm, respiratory failure, general cyanosis, loss of consciousness, foam on the lips, involuntary urination and defecation. The duration of an eclampsia attack can vary from a few minutes to hours. This form of spasmophilia is dangerous in terms of respiratory arrest or cardiac activity.

In children the first 6 months. life spasmophilia often occurs in the form of laryngospasm and eclampsia; at an older age - in the form of carpopedal spasm.

Diagnosis of spasmophilia

With an obvious form of spasmophilia, diagnosis is not difficult. The presence of clinical and radiological signs of rickets, complaints of muscle spasms and cramps are taken into account. To detect latent spasmophilia, appropriate mechanical or skin-galvanic tests are performed to assess neuromuscular excitability. A child with spasmophilia is examined by a pediatrician and a pediatric neurologist.

The diagnosis of spasmophilia is confirmed by a biochemical blood test that detects hypocalcemia, hyperphosphatemia, and other electrolyte imbalances; the study of CBS blood (alkalosis).

Differential diagnosis of spasmophilia should be carried out with other hypocalcemic conditions, convulsive syndrome, true hypoparathyroidism, epilepsy, croup, congenital stridor, whooping cough, etc.

Spasmophilia treatment

An attack of laryngospasm or eclampsia requires emergency care for the child, and in case of respiratory arrest and cardiac activity - resuscitation. To restore spontaneous breathing with laryngospasm, spraying the child's face with cold water, shaking it, patting the buttocks, irritating the root of the tongue, and providing fresh air can be effective. If ineffective, artificial respiration and chest compressions are performed, and humidified oxygen is supplied.

For any manifestations of obvious spasmophilia, the administration of anticonvulsants is indicated (diazepam intramuscularly, intravenously or into the root of the tongue, sodium oxybutyrate intramuscularly, phenobarbital orally or rectally in suppositories, chloral hydrate in an enema, etc.). Also shown in / in the introduction of 10% solution of calcium gluconate and 25% solution of magnesium sulfate in / m.

With a confirmed diagnosis of spasmophilia, a water pause is arranged for 8-12 hours, then, until the symptoms of latent spasmophilia disappear completely, the infants are transferred to natural or mixed feeding; older children are prescribed a carbohydrate diet (vegetable purees, fruit juices, tea with crackers, cereals, etc.).

To eliminate hypocalcemia, calcium gluconate is administered orally. After normalization of blood calcium levels and disappearance of signs of spasmophilia, anti-rachitic therapy is continued. In the recovery period, gymnastics, general massage, long walks in the fresh air are shown.

Forecast and prevention

Revealing and timely elimination of latent tetany allows avoiding the clinical manifestation of spasmophilia. With the provision of timely assistance for seizures and adequate correction of electrolyte disturbances, the prognosis is favorable. In extremely rare cases, in the absence of timely emergency care, the death of a child may occur due to asphyxia and cardiac arrest.

In its main provisions, the primary prevention of spasmophilia repeats the prevention of rickets. Breastfeeding plays an important role in preventive measures. Secondary prevention of spasmophilia consists in the treatment of the latent form of tetany, control of blood calcium levels, prophylactic calcium supplementation, and anticonvulsant therapy within 6 months after a seizure attack.

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