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Mounier-Kun syndrome



The clinical picture. From an early age cough with sputum, repeated exacerbations of bronchopulmonary disease, increase in respiratory failure during the period of exacerbation and with age. Deformation of the nail phalanxes in the form of "drum sticks".

Radiological signs: deformation of the pulmonary pattern with areas of consolidation. The expansion of the lumen of the trachea and large bronchi. Bronchiectasis in the lower lobe segments.

With bronchoscopy, the expansion of the lumen of the trachea (bronchi), thickening of the walls with bulging into the lumen of the interchondral spaces, pathological secretion.

Treatment. The treatment is conservative, aimed at fighting
with bronchopulmonary infection.

Monogenic lung disease:

1) primary ciliary dyskinesia and Cartegenera syndrome;

2) idiopathic diffuse pulmonary fibrosis (Hammen-Rich syndrome, idiopathic fibrosing alveolitis);

3) primary pulmonary hypertension (Aers syndrome);

4) idiopathic hemosiderosis of the lungs (Celen-Gellerstedt syndrome);

5) Goodpasture syndrome;

6) family spontaneous pneumothorax;

7) alveolar microlithiasis;

8) alveolar proteinosis;

9) cystic fibrosis;

10) insufficiency a ^ antitrypsin.

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Mounier-Kun syndrome

  1. 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
  2. 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
  3. 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
  4. 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
  5. 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.
  6. 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
  7. 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,
  8. 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
  9. 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
  10. 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
  11. 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
  12. 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
  13. 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
  14. Acute respiratory distress syndrome. Adult respiratory distress syndrome [distress]
    ICD-10 cipher J80 Diagnostics When making a diagnosis Mandatory Level of consciousness, respiration rate and efficiency, pulse, blood pressure, body temperature, history, physical examination R-graphy of the chest organs Blood gases Additional (if indicated) CVD DZLA During treatment Monitoring according to p.1.5 Additionally, the saturation of hemoglobin, hourly diuresis, blood gases,
  15. NEPHROTIC SYNDROME
    Nephrotic syndrome is a clinical and laboratory symptom complex characterized by proteinuria (more than 50 mg / kg / day or more than 40 mg / m2 / day, that is 3 g / day or more), hypoalbuminemia (less than 30 g / l), hyperlipidemia and swelling up to anasarca degree. ICD-10: N04 Nephrotic syndrome. Abbreviations: NA - nephrotic syndrome; BMI is a disease of minimal change; AH - arterial hypertension;
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