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Muscular dystrophy
- Progressive, genetically determined
- Degeneration of skeletal muscle, wasting, weakness
- Increased serum creatine kinase
- Duchenne’s muscular dystrophy is the most common type
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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
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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
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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
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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
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Rhabdomyosarcoma
most common soft tissue sarcoma in children - poor prognosis
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Peripheral neuropathy
diabetes is most common cause
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Atelectasis
- Collapsed lung
- Obstructive - compressive --- scarring - loss of surfactant
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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
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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
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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
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Bronchiectasis
- Permanent abnormal bronchial dilation
- Predisposed by bronchial obstruction (tumor) or chronic sinusitis
- Copious purulent sputum--can lead to lung abscess
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Restrictive Pulmonary disease
- Reduced expansion of the lung and reduced total lung capacity
- Abnormality of bony structures
- Neuromuscular disease
- Interstitial lung disease
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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
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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
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Lobar Pneumonia
- Most frequently caused by Strep
- Consolidation - may involve entire lobe
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Bronchopneumonia
- Patchy distribution
- Acute inflammation from bronchioles into adjacent alveoli
- Caused by variety of organisms: Staph aureus, Klebsiella, Haemophilus, Strep pyogenes
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Interstitial pneumonia (primary atypical)
- Diffuse, patchy inflammation
- Viruses of Mycoplasma pneumonia
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Asbestosis
- Pneumoconioses type
- Predisposition to mesothelioma and bronchogenic carcinoma; diffuse interstitial fibrosis; ferruginous bodies
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Anthracosis
- Type of Pneumoconioses
- Coal dust
- Carbon carrying macrophages
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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
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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)
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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.
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