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Goodpasture Syndrome



Goodpasture syndrome - a combination of hemosiderosis of the lungs and glomerulonephritis, is characterized by immunological damage to the basal membranes of the lungs and kidneys.

Pathogenesis. Erythrocytes for various reasons fall into the lung tissue from the vessels, become autoag; they generate autoAt; as a result of the reaction of AG-AT, the erythrocyte decay occurs. This hypothesis is based on the immunoallergic genesis of idiopathic hemosiderosis of the lungs. Refers to respiratory allergies, which can explain the cycle of the disease. Disease mainly of children's age.

Morphological picture: deposition of hemosiderin in the alveoli and interalveolar septa.

Clinical manifestations. Clinical picture: the onset of the disease can be in children of the first years of life; the course is usually wavy: periods of crises are replaced by remission of different duration. During the crisis, the temperature rises, coughing, shortness of breath, chest pain, hemoptysis (blood veins, intense sputum staining, possible bleeding), anemia; hepatolienal syndrome; gradually formed pulmonary heart.

Diagnostics.
In the blood test, hypergammaglobulinemia, increased CEC levels, detection of anti-pulmonary antibodies (sometimes), increased indirect bilirubin, hypochromic anemia, and decreased serum iron.

X-ray examination during the crises reveals multiple cloud-like shadows, usually bilateral; gradually formed pneumosclerosis.

Forecast. Patients die from pulmonary heart failure or pulmonary hemorrhage, spontaneous pneumothorax (due to thinning of the walls and rupture of subpleurally located emphysematous bullae). It refers to the number of hereditary diseases with an autosomal dominant type of inheritance (as an independent disease).

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Goodpasture Syndrome

  1. Goodpasture Syndrome
    Goodpasture's syndrome is excluded when combined with pulmonary hemorrhage and glomerulonephritis. Currently, his cause is considered to be the production of cytotoxic antibodies to the basal membrane of the glomerulus, caused by various factors (Goodpasture's syndrome has been described as a complication of influenza). It was shown that these antibodies bind to the alpha3 chain of type IV collagen. Men are more often ill at the age of 10-50 years.
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  3. NON-ANDROID-ENDOCRINE SYNDROME (HYPOTHALAMIC SYNDROME, METABOLIC SYNDROME)
    Different names of the neuro-exchange-endocrine syndrome (NOPS) are explained by several reasons. Etiology and pathogenesis of the syndrome are caused by hypothalamic disorders. In recent years, the genetic preconditions for the disease have been more clearly shown. Particular attention is paid to insulin resistance and the role of overweight in the development of such severe complications as insulin-dependent
  4. Abstract. Chromosomal sex diseases (Turner syndrome, trisomy X syndrome), 2009
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  5. Acute lung injury syndrome and acute respiratory distress syndrome
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  6. Syndrome of allergic conditions (hives, swelling of the quinck, Lyell syndrome)
    Allergic conditions requiring emergency care include: anaphylactic shock, urticaria, angioedema Quincke and Lyell syndrome. Anaphylactic shock, see the "Shock states syndrome" section. Urticaria Urticaria is an allergic disease characterized by the rapid spread of itching rashes on the skin, which are the edema of a restricted cutaneous area
  7. Syndrome (disease) of Bernard-Soulier (macrocytic thrombocytodystrophy, giant platelet syndrome)
    In this disease, a specific glycoprotein interacting with FV-VIII, FV, FIX and ristocetin is absent in the platelet membrane, and the content of sialic acids increases, and the electric charge decreases. This leads to a violation of the adhesion properties of platelets. The disease is inherited autosomally-recessively, characterized by a shortening of the life span of thrombocytes at their normal
  8. Acquired coagulopathy (disseminated intravascular coagulation, defibrillation syndrome, DIC syndrome)
    DIC-syndrome is a nonspecific general pathological process characterized by generalized activation of the hemostasis-anti-hemostasis system, at which the system of regulation of the aggregate state of blood occurs. The etiological factor of the disease are: - generalized infections, septic conditions; - shock of any origin; - extensive surgical interventions,
  9. Primary ciliary dyskinesia (syndrome of immobile cilia) and Kartagener's syndrome
    The basis is a genetically determined defect in the structure of the ciliated epithelium of the mucosa of the respiratory tract. The morphological essence of the defect in its classical version is reduced to the loss of the dineine handles containing ATP, which ensures the movement of cilia. The syndrome of Cartagena is characterized by the following triad, which includes the reverse location of internal organs, bronchiectasis and
  10. Amenorei-lactorea Syndrome (KIA-RI-FROMMEL SYNDROME)
    This syndrome also refers to postpartum or, less commonly, post-abortion complications. In the structure of violations of menstrual and generative function is found in 10% of observations. Pathogenesis is caused by functional hyperprolactinemia, which occurs during pregnancy as a result of hypertrophy of the pituitary lactotrophs, which is the physiological process necessary for lactation. Predisposing
  11. The syndrome of disseminated intravascular coagulation (DVS-syndrome)
    DIC-syndrome is a pathological condition that is determined by excessive intravascular coagulation and hemorrhagic consumption coagulopathy and manifests itself as a violation of the function of individual organs. The processes of intravascular coagulation are a natural attribute of the vital activity of the body. In the blood of every healthy person, like sick people, there are always those or
  12. Acute respiratory distress syndrome. Asthmatic status. Mendelssohn syndrome
    1. Patient S., 65 years old, suffers from bronchial asthma (hormone-dependent) for 15 years. The last attack of suffocation lasts the second day. Twice summoned an ambulance. In the department of resuscitation received in the state of asthmatic state (stage II). To name the diagnostic criteria of the II stage of status, to determine the sequence of IT. 2. Patient K., 40 years old, entered the surgical clinic
  13. Hemolytic-uremic syndrome (Gasser's syndrome)
    Hemolytic-uremic syndrome (HUS) was described by G. Gasser in 1956 in infants as a pathological syndrome characterized by a triad: acute hemolytic anemia, thrombocytopenia, acute renal failure. HUS develops more often in children under the age of 4 years. For adults, another, rarely occurring, pathology is characteristic: thrombotic, thrombocytopenic
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    This form of NOO refers to postpartum complications and is rare, as it develops as a result of inadequately conducted infusion therapy of massive bleeding after childbirth. Pathogenesis is caused by a decrease in the function of the pituitary gland due to circulatory disorders due to vasospasms in the background of DIC syndrome after massive bleeding or bacterial shock (complications of labor or
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