Temporal giant cell arteritis , cranial arteritis, temporal arteritis , Horton’s disease is a systemic lesion of arteries, mainly of muscular-elastic and muscle types.
In temporal giant cell arteritis, the vessels of the head (primarily the temporal arteries) and the thoracic aorta are most often affected. As a rule, elderly people are older than 50 years old, most often women. Several family cases of the disease are described. Temporal giant cell arteritis belongs to the group of allergic vasculitis .
The etiology is not known, possibly viral. There is a connection with respiratory infections, the content of birds (parrots) in the house, and the detection of hepatitis B surface antigen in serum.
In areas of vascular lesions, fixed immunoglobulin M and immunoglobulin G, components of the complement of the system, are detected. The acute phase is characterized by the phenomena of mucoid swelling, fibrinoid degeneration in the intima and middle lining of the arteries with thromboangiitis obliterans, necrotic changes and the transition to sclerosis. As part of the granulation tissue, giant multinuclear cells similar to Pirogov- Langgans cells are detected .
The onset of the disease is most often gradual – with the appearance of pain and stiffness in the neck, shoulder girdle, malaise, nausea, loss of appetite. An acute onset is also possible – high fever , sharp headaches in the area of the affected vessels, sometimes with impaired and loss of vision. A painful and throbbing temporal artery is palpated. In temporal giant cell allergy, there may be a combined lesion of the coronary and abdominal vessels.
There is a frequent combination of this disease and rheumatic polymyalgia ( Forestier – Sertonsini syndrome ), which consists in the appearance of symmetric polymyalgia in the elderly , especially in the neck muscles. shoulder girdle, and accompanied by low-grade fever, asthenia , anorexia.
In both cases, there is a sharp increase in ESR – from 50 to 100 mm / h, hyper-A2-globulinemia, hypoalbuminemia , moderate hyperchromic anemia.
There is a pronounced effect of glucocorticosteroid therapy (differential diagnostic sign of temporal giant cell arteritis). Symptom attenuation occurs within a few days after the administration of the glucocorticosteroid drug (initial dose of 40-50 mg, followed by a decrease to a maintenance dose of 5-10 mg). Long-term use of delagil , indomethacin is indicated , for relieving pain during an attack – novocaine chipping of near-arterial tissues.
Isolated pulmonary stenosis accounts for 1.6–9% of all congenital heart defects.
Anatomical variants of the defect are reduced to narrowing the path of outflow from the right ventricle of the heart. Distinguish valve stenosis (fusion of valve commissures); infundibular ( subvalvular ) stenosis due to the presence of a muscle diaphragm with a small opening or muscle hypertrophy of the exit tract of the right ventricle; supravalvular stenosis of the pulmonary trunk and branches of the pulmonary artery ( coarctation ). Sometimes there are combinations of the above variants of the anomaly.
The hemodynamics of the disease boils down to systolic overload of the right ventricle, which increases with increasing degree of stenosis. In this case, there is a restriction of pulmonary blood flow, which determines the degree of cyanosis. Compensation of the defect is supported by hypertrophy of the right ventricle. Characteristically, dila – tation it usually does not happen. Thus, hypertrophic syndrome of the “small ventricle” develops. Due to the increase in the rate of its reductions, as a rule, due MOS is kept relatively long. Over time, ischemic contracture of the hypertrophied myocardium of the right ventricle develops and it ceases to cope with a high load. Decompensation sets in: cyanosis appears, venous pressure rises, hepatomegaly , peripheral edema develop and progress .
The clinic is determined by the severity of stenosis and the state of compensatory mechanisms. Clinical manifestations range from asymptomatic to severe forms (severe shortness of breath at the slightest exertion, anginal pain, cyanosis). Auscultation is characterized by gross systolic murmur with an epicenter in the second and third intercostal space on the left ( infundibular stenosis). In the late stages of the defect, cyanosis, venous hypertension (visible dilatation of the cervical veins), enlargement of the liver, and peripheral edema join.
Radiologically observed: a moderate increase in the right ventricle, narrow roots of the lungs, impoverishment of the pulmonary pattern. Valvular stenosis is characterized by suprastenotic expansion of the pulmonary artery due to hydrodynamic shock of a narrow stream of blood. On the ECG – a rightogram , signs of systolic overload of the right ventricle. FCG reveals a rhomboid systolic murmur in the pulmonary artery, a decrease in the amplitude of II tone in this zone. To determine the severity of stenosis and clarify indications, surgical treatment is resorted to sounding of the heart cavities. The main clinical criteria are the systolic pressure gradient between the right ventricle and the pulmonary artery, as well as the systolic pressure in the right ventricle. The latter criterion is more informative. It determines the clinical stages of the course of the disease: I – moderate stenosis (systolic pressure in the right ventricle below 60 mm Hg (8 kPa); II – severe stenosis (systolic pressure in the right ventricle 60 to 100 mm Hg (8 —13.3 kPa), III – severe stenosis (systolic pressure in the right ventricle is higher than 100 mmHg (13.3 kPa). With coarctation of the pulmonary trunk and its branches, angiopulmonography is most informative .
The remains of a hyaloid artery is an anomaly of development that occurs due to a violation of the reverse development of the hyaloid artery in the embryonic period. Usually found at a young age, with age, this artery resolves. The clinical picture and diagnosis. It manifests itself in the form of a strand (moorings) of the vitreous body, which goes from the optic disc to the lens. More often, the remains of an artery are detected in the anterior or posterior parts, and not along the entire length. In this case, the artery has the form of a corkscrew or rope, the end of which floats freely in the vitreous. Very rarely, the remains of the hyaloid artery are shrouded in a layer of connective tissue and can significantly impair vision.
Thrombosis is a pathological condition characterized by the formation of a blood clot in the area of the vascular bed.
Indispensable conditions for the occurrence of arterial thrombosis is a violation of the integrity of the vascular wall, changes in the hemostatic system and a slowdown in blood flow. This explains the high frequency of thrombosis in people suffering from obliterating atherosclerosis, diabetes mellitus. Often the development of thrombosis contribute to damage to the walls of the arteries. soft tissue bruises, dislocations and fractures of limb bones, compression of the vascular bundle by a tumor or hematoma. Acute arterial thrombosis may be preceded by angiography – cal research, reconstructive vascular surgery, intra-arterial blood transfusion. Thrombosis also occurs against the background of certain hematologic (polycythemia) and infectious (typhus) diseases.
In all these cases, the response to damage to the endothelium of the vascular wall is adhesion and subsequent platelet aggregation. The resulting aggregates tend to further growth, which is associated with the action of physiologically active substances released from platelets. Released from platelets trombotsitarnge factors and biologically active substances contribute not only platelet aggregation, but also the activation of the blood coagulation system, reducing its fibrinoli – cal activity. Thus, thrombocytic aggregates become a functionally active field in which the activation and interaction of coagulation factors of the blood occurs. As a result, fibrin filaments are adsorbed on the surface of the aggregate and a network structure is formed, which, delaying the formed elements of the blood, turns into a blood clot. With significant inhibition of the lytic link of the hemostatic system, thrombosis can take on a common character.
Temporal arteritis (giant cell arteritis, Horton’s disease ) – a disease of the elderly and senile, more common in women, characterized by damage to the aorta and arteries of the head and neck. Rheumatic polymyalgia is a disease of people of the same age and gender, characterized by pain and stiffness in the proximal parts of the muscles of the shoulder and pelvic girdle in combination with fever, weight loss. Both diseases are often found in the same patient, and therefore their common origin is not excluded, the role of a viral infection, including hepatitis B virus, is possible. Temporal arteritis is characterized by granulomatous inflammatory infiltrates with lymphocytes and giant multinucleated vessels in the vessels extending from the aortic arch; more often the temporal arteries are affected. Gradually, a thickening of the inner membrane develops with a narrowing of the lumen of the vessels and parietal thrombosis. The most common symptom is a burning throbbing headache. The temporal arteries are painful and swollen, the skin above them is hyperemic. Subsequently, the temporal arteries condense, become crimped. The most serious complications are eye damage, 20% of patients develop vision loss due to ischemia of the optic nerves. Intermittent pain in the masticatory muscles, sometimes in the muscles of the tongue and the muscles involved in swallowing, can be observed . In 10-15% of patients, large arteries are affected, ischemic pains in the upper or lower extremities, noises or a decreased pulse on the vessels of the neck or limbs, sometimes aortic rupture are noted. More rare symptoms are cough, depression, psychosis, hemiparesis, peripheral neuropathy , acute hearing loss, cerebrovascular accident, myocardial infarction. Most patients have a fever, sometimes of an unclear origin. Almost all patients note a sharp weakness, weight loss, anorexia. Half of the patients have signs of rheumatic polymyalgia – pain and stiffness in the muscles of the shoulder and pelvic girdle, neck. Often there is moderate normochromic anemia, thrombocytosis. The white blood cell count is usually normal. A significant increase in ESR is characteristic. In 1/3 of patients, moderate liver dysfunction is detected. The diagnosis of temporal arteritis should be discussed in any patient older than 50 years who has a headache, transient or sudden loss of vision, pain and stiffness in the muscles, prolonged fever, anemia, and an increase in ESR. The diagnosis is confirmed by biopsy of the temporal artery. Differentiate temporal arteritis with periarteritis nodosa, which can affect the temporal arteries, nonspecific aortoarteritis , RA, vasculitis , migraine, trigeminal neuralgia, intracranial pathological processes, including tumors. After diagnosis, glucocorticoids should be prescribed immediately (prednisone at a dose of 45-60 mg per day). After a marked improvement in clinical and laboratory signs, the dose can be slowly reduced (2.5-5 mg every 2-4 weeks ). Long-term maintenance therapy is indicated, however, in most patients, glucocorticoids can subsequently be canceled. The prognosis is usually favorable , the disease does not affect life expectancy. The main serious complication is blindness. The prognosis worsens with damage to large vessels and aorta. The main clinical sign of rheumatic polymyalgia is pain in the muscles of the shoulder and pelvic girdle. Morning stiffness, as well as stiffness after a period of immobility, restriction of movements in the cervical spine, shoulder and hip joints are characteristic . Patients hardly get out of bed, dress, comb their hair, rise and go down the stairs; the gait becomes minced. Muscle strength usually does not decrease, although atrophy from inaction (due to pain ) can develop . With a careful examination of the joints, the detection of synovitis is possible . Pain in the proximal joints, tendons, and joint bags may be noted. Unpleasant sensations may appear first on one side, but soon become bilateral, common symptoms are characteristic – weakness, moderate fever, weight loss. Some patients have signs of temporal arteritis, primarily a headache, which may be the first sign of the disease. ESR is sharply increased, sometimes up to 60-80 mm / h. Mild normochromic anemia may be detected . Increased levels of fibrinogen, C-reactive protein. Polymyalgia rheumatica should be considered when there are severe bilateral pains in the muscles of the neck, shoulder and pelvic girdle, accompanied by a significant increase in ESR in patients over the age of 50 years. The disease should be differentiated with systemic vasculitis , dermatomyositis, RA, paraneo -plastic syndrome.
Lowering blood pressure in children is quite common (from 5.8 to 14.8% – V.N.; 3asukhina and E.V. Fedorova), and it is often underestimated in terms of health status. Arterial hypotension is primary and secondary: with cardiovascular, endocrine, infectious diseases, against the background of nutritional disorders and dystrophy.
In the development of arterial hypotension, a hereditary predisposition, a violation of the function of the endocrine glands (neurohumoral dysregulation ) is important . A major role is played by foci of chronic infection (tonsillitis, sinusitis, cholecystocholangitis , dental caries).
The basis of the changes are corticovisceral disorders, leading to a drop in blood pressure (neurocirculatory dystonia).
In most cases, children do not complain, although they periodically have a decrease in blood pressure, headache, excessive sweating, irritability, fatigue, periodic dizziness and fainting. The pulse is labile. From the side of the heart – muffled I tone, systolic murmur of a functional nature. Blood pressure is lowered, initially systolic, then diastolic can also decrease. These changes may be transient, less often they become persistent.
Arterial hypotension should be differentiated with various chronic intoxications and the latent course of rheumatism. Secondary hypotension must be excluded.
The prognosis is favorable in most children with a transient decrease in blood pressure. However, the disease often proceeds for a long time, periods of improvement are replaced by periods of deterioration (under the influence of various adverse factors). With secondary hypotension, the prognosis depends on the course of the underlying disease.
The correct regime of the day, the maximum stay in the fresh air, physical education, water procedures. Of great importance is a nutritious diet with sufficient intake of vitamins. In severe cases, you can prescribe caffeine, tincture of ginseng, Schisandra chinensis, zamanicha, aralia, pantocrine, bromides, tincture of valerian, elenium, andaxine . Physiotherapeutic procedures are applied with a tonic effect on the body. It is necessary to treat a focal chronic infection (debridement). With secondary hypotension, rational therapy of the underlying disease is of great importance.
The correct regime of the day, a combination of training with physical education and sports, hardening procedures.
Obliterating brachiocephalic arteritis (synonyms: pulseless disease, Takayasu’s disease ) is a non-specific inflammatory lesion of the aortic wall and large major vessels with their subsequent obliteration. Obliterating brachiocephalic arteritis has been described by Japanese physician Takayasu . Most often, young women get sick. The etiology is not known. A connection with repeated infections (tonsillitis, flu, typhus), hypothermia, trauma, and pregnancy is assumed.
Obliterating brachiocephalic arteritis belongs to the group of allergic vasculitis . The hyperergic nature of inflammation is confirmed by the presence of immune complexes at the site of the lesion and anti-arterial antibodies in the serum.
With obliterating brachiocephalic arteritis, panarteritis is observed with an initial lesion of adventitia and intima with their exudative infiltration, disorganization, spreading to the middle membrane, subsequent fibrinoid organization, sclerosis and the development of parietal thrombosis.
The disease is slowly progressive in nature. Most often, obliterating brachiocephalic arteritis begins with common symptoms: malaise, fever, arthralgia, night sweat, gradual asthenia . As the limbs ischemic , a feeling of numbness, an increase in weakness in the arm, a one-sided or symmetrical absence of a pulse on the affected limbs appear. Often “eye” symptoms (vision loss, pain in the eyes) associated with the formation of arteriovenous retinal aneurysms and optic atrophy join in . In most cases, hypertension associated with damage to the abdominal aorta and renal arteries is noted. Less characteristic (up to 20% of cases) damage to the pulmonary and coronary vessels. With obliteration of the carotid arteries, neurological symptoms associated with cerebral ischemia develop . Laboratory data are not characteristic. Possible anemia, ESR increased to 50 mm / h. Factor rheumatoid and antinuclear antibodies are not determined. System complement components are normal. The diagnosis is confirmed by arteriography .
Long-term administration of glucocorticosteroid drugs, indomethacin , delagil , drugs that improve peripheral blood flow ( trental , compliance , angiotrophip , prodectin ), heparin and indirect anticoagulants is indicated . In recent years, reconstructive surgery of affected vessels has been proposed.
This defect is based on the abnormal position of the ascending aorta and the trunk of the pulmonary artery. There are 2 main types of this anomaly: complete and corrected transposition of the aorta and pulmonary artery. Complete transposition of the great vessels makes up 4.5–20.8% of all congenital heart defects. Together with tetralogy of Fallot, this vice takes a leading place among the “blue” vices. Its hemodynamics boils down to the fact that the aorta departs from the anatomically right ventricle, and the pulmonary artery from the left. As a result, blood circulation occurs in two divided circles. In the absence of communication between them, the newborn dies immediately after birth due to the lack of conditions for blood oxygenation . Therefore, a prerequisite for viability is communication between these independent circles of blood circulation. Usually they are represented by defects of the septa, OAA, aortopulmonary fistula, abnormal venous drainage, or combinations thereof. In this case, cross blood circulation is formed, which determines the severity of the course of the defect. It is determined by the degree of overflow of one and “robbing” of another circle of blood circulation. The heart in these conditions works with overload. Nevertheless, the larger the shunt, the more favorable the course of the defect. So, the most favorable combination of septal defects with moderate stenosis of the pulmonary artery, which prevents excessive pulmonary blood flow. Corrected transposition of the great vessels makes up 1–1.4% of all congenital heart diseases. In this case, the aorta and pulmonary artery are transposed, but the right ventricle receives blood from the left atrium, and the left from the right. Thus, arterial blood enters the large circle of blood circulation, and venous blood enters the small circle. If there are no other concomitant anomalies, hemodynamic disturbances are absent.
Shortness of breath, cyanosis, fatigue, finger changes in the form of drumsticks, peripheral circulatory disorders, lag in physical development are noted. The auscultatory picture is diverse and is determined by the shape of the shunt between the circles of blood circulation. Along with the presence of pronounced systolic murmur along the left edge of the sternum, noise is often absent.
X-ray examination early reveals cardiomegaly due to both parts of the heart. More often the heart has a spherical shape. There is an increase in vascular pattern of the lungs, pronounced vascular pulsation on the periphery. An ECG allows you to identify only signs of overload of various parts of the heart, single-beam blockade of the conduction system. The most complete information about anatomical and hemodynamic malformations is provided by Doppler ultrasound, heart sounding, and angiocardiography .
Hereditary hemorrhagic telangiectasia (synonyms of the disease: Rendu- Weber- Osler disease) is a disease caused by thinning and dilatation of the walls of arterioles and capillaries. Hereditary hemorrhagic telangiectasia is transmitted by an autosomal dominant type, manifested in adults. The alleged defect is associated with impaired collagen synthesis in the vascular wall. Patients on the skin and mucous membranes of the nose, mouth, and gastrointestinal tract have characteristic vascular “spiders” of red color that disappear with pressure, which later rise above the surface and form nodules; in the lungs, arteriolovenous aneurysm of ism develops . Rupture of a dilated vessel (with mechanical compression, spontaneous) can cause hemorrhages.