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Goodpasture syndrome



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

Pathogenesis. Erythrocytes for various reasons enter the lung tissue from the vessels, become autoAG; they produce autoAT; as a result of the AG-AT reaction, erythrocyte disintegration occurs. This hypothesis is based on the immunoallergic genesis of idiopathic pulmonary hemosiderosis. It refers to respiratory allergies, which can explain the cyclical nature of the disease. The disease is predominantly of childhood.

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

Clinical manifestations. Clinical picture: the onset of the disease may be in children of the first years of life; the course is usually wave-like: periods of crises are replaced by remission of various duration. During crises, fever, cough, shortness of breath, chest pain, hemoptysis (blood streaks, intensive sputum staining, pulmonary hemorrhage), anemia; hepatolienal syndrome; gradually formed pulmonary heart.

Diagnostics.
In the analysis of blood hypergammaglobulinemia, increased levels of the CEC, the identification of anti-pulmonary antibodies (sometimes), an increase in indirect bilirubin, hypochromic anemia, a decrease in serum iron.

X-ray examination in the period of crises revealed multiple cloud-like shadows, usually bilateral; gradually formed pneumosclerosis.

Forecast. Patients die from pulmonary heart disease or pulmonary hemorrhage, spontaneous pneumothorax (caused by thinning of the walls and rupture of subpleurally located emphysematous bull). 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 syndrome is excluded with a combination of pulmonary hemorrhage and glomerulonephritis. Currently, its cause is considered to be the production of cytotoxic antibodies to the glomerular basement membrane, due to various factors (Goodpasture syndrome has been described as a complication of influenza). These antibodies have been shown to bind to the alpha3-chain of type IV collagen. More often men at the age of 10 - 50 years are ill.
  2. Syndrome of premature arousal. Lowna-Ganonga-Levin syndrome. Wolff-Parkinson-White syndrome
    Code for ICD-10 I45.6 Diagnosis When making a diagnosis Mandatory Level of consciousness, frequency and effectiveness of breathing, heart rate, pulse, blood pressure, ECG, if possible, a minimum history In the process of treatment Monitoring in accordance with clause 1.5. After the restoration of the rhythm - ECG in the dynamics, hourly diuresis, Laboratory studies: hemoglobin, blood gases, KOS indicators, electrolytes
  3. NON-EXCHANGE-ENDOCRINE SYNDROME (HYPOTALAMIC SYNDROME, METABOLIC SYNDROME)
    Different names of neuroexchange-endocrine syndrome (NNPP) are explained by several reasons. The etiology and pathogenesis of the syndrome are caused by hypothalamic disorders. In recent years, the genetic background to the disease is more clearly shown. Particular attention is paid to insulin resistance and the role of overweight in the development of such serious complications as insulin-dependent
  4. Abstract. Chromosomal diseases of sex (Turner syndrome, trisomy X syndrome), 2009
    Introduction Mechanism of Developmental Disorders in Chromosomal Diseases Classification of Chromosomal Diseases General Characteristics of DiseasesShereshevsky-Turner Syndrome Trisomy Syndrome-X Conclusion List Used
  5. Syndrome of acute lung injury and acute respiratory distress syndrome
    Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are non-specific damage to the lung parenchyma of a polyetiological nature and are characterized by: • ????? acute onset; • ???? progressive arterial hypoxemia; • ???? bilateral infiltration of the pulmonary fields on the radiograph of the chest; • ???? progressive decline
  6. Allergic States Syndrome (Urticaria, Quinck Edema, Layel Syndrome)
    Allergy conditions that require emergency care include: anaphylactic shock, urticaria, angioedema, angioedema, angioedema, angioedema, and layel syndrome. Anaphylactic shock, see "The syndrome of shock states." Urticaria Urticaria is an allergic disease characterized by the rapid spread of itchy lesions on the skin, which is a swelling of a limited skin area.
  7. Syndrome (disease) Bernard - Soulier (macrocytic platelet dysfunction, giant platelet syndrome)
    In this disease, the platelet membrane lacks a specific glycoprotein that interacts with PV-VIII, FV, FIX and ristocetin, as well as increases the content of sialic acids, decreases the electrical charge. This leads to a violation of the adhesive properties of platelets. The disease is inherited autosomal recessively, characterized by shortening of the life span of platelets at their normal
  8. Acquired coagulopathy (disseminated intravascular coagulation, defibrination syndrome, DIC)
    DIC syndrome is a non-specific general pathological process characterized by generalized activation of the hemostasis-anti-hemostasis system, during which there is a mismatch in the regulation systems of the aggregative state of the blood. The etiological factor of the disease are: - generalized infections, septic conditions; - shock of any origin; - extensive surgical interventions,
  9. Primary ciliary dyskinesia (immotile cilia syndrome) and Cartagener syndrome
    It is based on a genetically determined defect in the structure of the ciliated epithelium of the mucous membrane of the respiratory tract. The morphological nature of the defect in its classic version is reduced to the loss of dyneinovyh pens containing ATP, which ensures the movement of cilia. Carthage's syndrome is characterized by the following triad, which includes the reverse arrangement of internal organs, bronchiectasis and
  10. SYNDROME OF AMENORE-LACTOR (KIA-RI-FROMMEL SYNDROME)
    This syndrome also applies to postpartum or less commonly post-sided complications. In the structure of violations of the menstrual and generative functions found in 10% of observations. The pathogenesis is due to functional hyperprolactinemia, which occurs during pregnancy as a result of hypertrophy of the pituitary lactotrophs, which is a physiological process necessary for lactation. Predisposing
  11. Disseminated intravascular coagulation syndrome (DIC)
    DIC syndrome is a pathological condition that is determined by excessive intravascular coagulation of blood and hemorrhagic coagulopathy of consumption and is manifested by dysfunction of individual organs. The processes of intravascular coagulation are a natural attribute of the vital activity of the organism. In the blood of every healthy person, like sick people, there are always those or
  12. Acute respiratory distress syndrome. Asthmatic status. Mendelssohn's syndrome
    1. Patient S., 65 years old, suffers from bronchial asthma (hormone-dependent) for 15 years. The last attack of suffocation continues for the second day. Twice she called an ambulance. She was admitted to the intensive care unit in a state of asthma (stage II). Call the diagnostic criteria stage II status, determine the sequence of IT. 2. Patient K., 40 years old, was admitted to the surgical clinic
  13. Hemolytic uremic syndrome (Gasser syndrome)
    Hemolytic uremic syndrome (HUS) was described by G. Gasser in 1956 in infants as a pathological syndrome characterized by a triad: acutely developing 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 characterized - thrombotic, thrombocytopenic
  14. POSTBIRTH HYPOPUITITARIANISM SYNDROME (SHIENE SYNDROME)
    This form of NNPP is related to postpartum complications and is rare since it develops as a result of inadequately performed infusion therapy for massive bleeding after childbirth. The pathogenesis is due to a decrease in the function of the pituitary gland as a result of circulatory disorders due to vascular spasms on the background of DIC after massive bleeding or bacterial shock (complications of labor or
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