Systemic pathology 400 (Respiratory pathologies 1-2) Flashcards
(225 cards)
SSx – potential SSx for respiratory disorders
wheezing, rales
lightheadedness
thicker mucus
cough
shortness of breath (dyspnea)
Dx – potential Dx for respiratory disorders
auscultation
imaging
pt Hx
physical exam
spirometer
biopsy (?)
Tx – potential Tx for respiratory disorders
bronchodilator
anti-inflammatories
Cystic fibrosis
inherited genetic disorder
AUTOSOMAL RECESSIVE
–> most fata autosomal recessive disorder
Affects EXOCRINE glands
–> including pancreas glands
–> and bronchial mucus glands
CF affecting exocrine glands
Defective ion transport channels of Na+ and Cl-
Mucosal secretions become very thick and viscous
which systems are most affected by CF
Respiratory,
digestive,
and reproductive systems are most affected
what does CF predispose individual to?
Predisposes to chronic bacterial airway infections
what does CF progressively lead to?
Progressive loss of pulmonary function
CF incidence – which groups most common in>?
Most common inherited genetic disease in white Europeans
how many new cases are diagnosed per year in US?
Over 1000 new cases diagnosed per year (US)
how many people are carriers of CF?
~1 in 25 are carriers
CF etiology
Autosomal recessive
which chromosome is affected in CF?
Genetic defect on chromosome 7
chromosome 7 affected d/t …?
Spontaneous mutation
CF pathogenesis
Impaired Na+/Cl- channels cause accumulation of salt in tissues
Surrounded mucous is abnormally thick
CF mucus salt concentrations are …
People with cystic fibrosis have a higher than normal level of salt in their sweat
mucous gland secretions viscosity is …?
Dehydrated and INCREASED viscosity of mucous gland secretion
note sweat glands and CF (sweat glands are also exocrine glands)
Elevation of sweat electrolytes (sodium chloride)
CF sweat test (Dx)
A chloride sweat test helps diagnose cystic fibrosis (CF), an inherited disorder that makes kids sick by disrupting the normal function of epithelial cells.
These cells (exocrine cells?) make up the sweat glands in the skin and also line passageways inside the lungs, liver, pancreas, and digestive and reproductive systems.
CF and pancreas
Pancreatic enzyme insufficiency
why pancreatic enzyme insufficiency?
Mucus also collects in the pancreas, which keeps digestive enzymes from reaching the small intestine.
result of pancreatic enzyme insufficiency?
Leads to malabsorption of nutrients
Stools are bulky, greasy and malodorous
Children often show growth abnormalities
most important complicaiton of CF
Most important complication involves bronchial mucosa
Bronchial mucous plugs prevent normal respiration
CF clinical manifetations
Variable