Pathology Final - Muscle, Resp

  1. Muscular dystrophy
    • Progressive, genetically determined
    • Degeneration of skeletal muscle, wasting, weakness
    • Increased serum creatine kinase
    • Duchenne’s muscular dystrophy is the most common type
  2. Duchenne's muscular dystrophy
    • Deficiency of dystrophin, a plasma membrane protein providing functional integrity of muscle cells
    • X linked; 33% represent new mutations however
    • Weakness initially in proximal extremity muscles and pelvic girdle
    • Pseudohypertrophy of calf muscles
    • Gower’s sign
    • Death in teens, early 20s
  3. Myotonic dystrophy
    • Autosomal dominant, triple nucleotide repeat
    • Symptoms begin as adult—weakness of hands and foot muscles initially
    • Hatchet face expression
    • Associated with increased frequency of cardiomyopathy, cataracts, and diabetes
  4. Myasthenia gravis
    • Autoantibodies to acetylcholine receptors
    • Women (20-35 years old) Men (50-60 years old)
    • Muscle weakness intensified by muscle use—worse later in the day
    • 15% associated with tumors of the thymus; 65% with thymic hyperplasia
    • Ocular muscle droop (ptosis), diploplia (double vision), inability to chew
  5. Polymyositis
    • Chronic degenerative autoimmune process involving proximal muscles of extremities—weakness and pain walking up stairs
    • Can be associated with other autoimmune diseases
    • Called Dermatomyositis when it also involves the skin---heliotrope (lilac rash) discoloration around the eyes
    • Muscle biopsy shows necrotic muscle surrounding by lymphocytes and plasma cells
  6. Rhabdomyosarcoma
    most common soft tissue sarcoma in children - poor prognosis
  7. Peripheral neuropathy
    diabetes is most common cause
  8. Atelectasis
    • Collapsed lung
    • Obstructive - compressive --- scarring - loss of surfactant
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  9. COPD (chronic obstructive pulmonay disease)
    • Characterized by airflow obstruction
    • AKA - Bronchial asthma: Bronchial smooth muscle, Hypertrophy/hyperplasia of submucosal glands and goblet cells; mucus plugging containing Curshmann spirals, eosinophils, and Charcot-Leyden crystals o
    • Includes both allergic (extrinsic, immune) (type I hypersensitivity) and intrinsic (non-immune) asthma (associated with chronic bronchitis or exercise/cold induced asthma),dyspnea and wheezing, Intrinsic usually begins in adult life whereas extrinsic usually begins in childhood
  10. Chronic bronchitis
    • Productive cough for at least 3 consecutive months over 2 consecutive - cigarette smoking, air pollution; hyperplasia of mucous glands
    • May lead to cor pulmonale
  11. Emphsysema
    • Abnormal dilation of air spaces with destruction of alveolar walls
    • Loss of elastic recoil
    • Clinically associated with increased total vital capacity, increased dimension of the chest, hypoxia, cyanosis (if assoc. with chronic bronchitis), respiratory acidosis
    • Strongly associated with cigarette smoking 1. Centrilobular--bronchioles dilated—upper lobes 2. Panacinar--entire acinus--can be deficiency of alpha 1 antitrypsin; distributed uniformly throughout lung
  12. Bronchiectasis
    • Permanent abnormal bronchial dilation
    • Predisposed by bronchial obstruction (tumor) or chronic sinusitis
    • Copious purulent sputum--can lead to lung abscess
  13. Restrictive Pulmonary disease
    • Reduced expansion of the lung and reduced total lung capacity
    • Abnormality of bony structures
    • Neuromuscular disease
    • Interstitial lung disease
  14. Adult Respiratory distress syndrome (ARDS)
    • Produced by diffuse alveolar damage (DAD) Leakage of protein rich fluid into alveoli
    • Formation of intra-alveolar hyaline membrane
    • Caused by shock, sepsis, trauma, uremia, aspiration of gastric contents, inhalation of chemical irritants, heroin overdose, acute pancreatitis
  15. Neonatal respiratory distress syndrome (hyaline membrane disease)
    • Most common cause of respiratory failure of the newborn
    • Most common cause of death with premature infants
    • Results from surfactant deficiency
    • Heavy lungs; hyaline membranes; intraventricular brain hemorrhage; necrotizing enterocolitis
  16. Lobar Pneumonia
    • Most frequently caused by Strep
    • Consolidation - may involve entire lobe
  17. Bronchopneumonia
    • Patchy distribution
    • Acute inflammation from bronchioles into adjacent alveoli
    • Caused by variety of organisms: Staph aureus, Klebsiella, Haemophilus, Strep pyogenes
  18. Interstitial pneumonia (primary atypical)
    • Diffuse, patchy inflammation
    • Viruses of Mycoplasma pneumonia
  19. Asbestosis
    • Pneumoconioses type
    • Predisposition to mesothelioma and bronchogenic carcinoma; diffuse interstitial fibrosis; ferruginous bodies
  20. Anthracosis
    • Type of Pneumoconioses
    • Coal dust
    • Carbon carrying macrophages
  21. Sarcoidosis
    • Restrictive lung disease of unknown etiology
    • Non-caseating granulomas
    • Multiple organ systems
    • Young adults; Black patients;
    • Bilateral hilar lymphadenopathy
    • Interstitial lung disease
    • Hypercalcemia, hypercalciuria
    • Elevated serum angiotensin-converting enzyme
  22. Cystic fibrosis
    • Characterized by production of thick mucus
    • Most common autosomal recessive
    • 1 in 25 caucasians are heterozygous carriers
    • Can cause obstruction in lungs (bronchi), intestines (meconeum ileus), and pancreas (malabsorption)
  23. Bronchogenic carcinoma
    • (cigarette related usually although can be radiation related)
    • 5 year survival less than 10%
    • Squamous cell---can have inappropriate parathyroid-like activity
    • Small cell (oat cell) ---very aggressive may have ectopic production of ACTH or ADH (paraneoplastic syndrome)—not amenable to surgery
    • Adenocarcinomas—slowest growth rate; equal sex predilection as contrasted to the other types of lung cancer which are seen more often in males.; Typically a PERIPHERAL location—most often due to second hand smoke exposure.
Card Set
Pathology Final - Muscle, Resp
Pathology Final