Term
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Definition
Chronic pulmonary disease
Pancreatic exocrine insufficiency
Complications of viscous secretions in other organs including the small intestine, liver and reproductive organs |
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Term
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Definition
| multiple chromosomal mutations |
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Term
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Definition
| not diagnostic of cystic fibrosis |
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Term
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Definition
| diagnostic of cystic fibrosis |
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Term
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Definition
| pancreatic enzyme replacements need to be eaten with all meal and snacks (can be sprinkled over food) |
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Term
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Definition
| failure of the sweat glands to resorb sodium and chloride |
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Term
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Definition
Cough
Repeated pneumonia
Wheezing
Clubbing of digits
Nasal polyps
Sinusitis
Cyanosis
Fatty stools |
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Term
| high caloric and high protein diet |
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Definition
| what kind of diet do you need with CF? |
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Term
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Definition
| Hgb S is subsituted for A |
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Term
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Definition
Single base mutation in the 6th codon (GAG to GTG) of the beta-hemoglobin gene
Results in substitution of valine for glutamic acid in beta chain ( always only one amino acid out of place, just one) |
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Term
| 16-20 (normal RBC lasts 120 days) causing chronic anemia |
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Definition
| sickle cells last only... |
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Term
| Vaso-occlusive crisis (acute pain) |
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Definition
| Cells sickle; plug up capillaries |
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Term
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Definition
| infection causes bone marrow to shut down and stop creating RBC’s and causing them to be very anemic |
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Term
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Definition
| splenic infarction; blood will come into the spleen and wont be able to get out; spleen distends; life threatening |
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Term
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Definition
| severe respiratory distress; end up in ICU on ventilators many times |
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Term
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Definition
| Repetitive injuries of a severe nature |
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Term
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Definition
| All states passes child abuse laws |
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Term
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Definition
| Form of child abuse in which the caretaker, usually the mother, systematically fabricates information about a child’s health or intentionally makes the child ill in order to meet own needs |
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Term
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Definition
Chronic inflammatory disorder of the airways that
Involves a variety of cells (including mast cells, eosinophils, T lymphocytes, macrophages, neutrophils and epithelial cells) and cellular mediators
Results in recurrent episodes of reversible airflow obstruction causing wheezing, breathlessness, cough |
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Term
Inflammation
Bronchospasm
Edema
Hyper-secretion of mucous |
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Definition
| reversible changes in asthma include: |
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Term
Damage/loss of airway epithelium
Collagen deposit below basement membrane causing thickening
Increased vascularity and inflammation
Loss of elastic fibers
Smooth muscle thickening(? hyperplasia or hypertrophy) |
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Definition
| Long term (irreversible changes) in the airway of asthma include: |
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Term
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Definition
| prolonged asthma that responds poorly to treatment |
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Term
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Definition
| fulminant form of asthma with rapid and unexpected progression from apparently controlled asthma to death from respiratory arrest |
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Term
Peak Expiratory Flow Rate (PEFR)
<50%=moderate obstruction
<25%=severe obstruction |
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Definition
| simple way to obtain an objective assessment of the degree of obstruction (asthma) |
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Term
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Definition
Short acting inhaled beta 2 agonists
Systemic corticosteroids (oral prednisone is the one you mostly use)
Anticholinergics
If you are using these more than twice a week than your long term controllers aren’t doing their job |
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Term
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Definition
Inhaled corticosteroids
Long acting beta 2 agonists
Cromolyn (don’t use much anymore), nedocromil
Leukotriene modifiers (important; singulair?)
Methylxanthine |
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Term
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Definition
| Often present with hoarseness, stridor, barking cough |
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Term
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Definition
Acute onset. May have mild preceding URI
Fever, tachypnea and tachycardia
Sore throat, dysphagia, drooling
Inspiratory stridor, muffled voice
Retractions
Sit leaning forward (“sniffing position”)
Child anxious and agitated
Toxic appearance--appear sicker than they sound |
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Term
| Acute Laryngotracheobronchitis |
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Definition
Viral infection that results in edema and inflammation in the glottic and sub-glottic areas of the airway
Because the child’s airway is narrower than an adult’s, a given amount of swelling reduces the diameter of the child’s airway proportionately more than in an adult |
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Term
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Definition
Life-threatening infection that may be preceded by croup
Child appears toxic with fever and increasing respiratory distress unrelieved by nebulized epinephrine
Soft tissue radiographs show ragged appearance of tracheal wall (like when you tear a paper.. That is what the tracheal wall looks like)
Requires prompt recognition, IV antibiotics, intubation, frequent suctioning to prevent acute obstruction from purulent secretions (keep suctioning because secretions are very purulent) |
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Term
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Definition
Common viral illness
Causes inflammation of small airways (effects the small terminal bronchioles)
Characterized by wheezing
Mostly caused by RSV |
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