Systemic pathology 400 (Respiratory pathologies 1-2) Flashcards
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
severity of SSx/clinical manifestations of CF associated strongly with …
Severity strongly associated
with socioeconomic status
and access to healthcare
important clinical manifestation (as mentioned above as well)
sweat/skin
Abnormally high sodium and chloride concentrations in
the sweat
CF GI tract manifestations
Poor nutrition (malabsorption)
Weight loss
Growth retardation/FTT (failure to thrive in newborns)
Intestinal obstruction from thick, dry stool
Prolapse of the rectum (??)
why prolapse of rectum during CF
Rectal prolapse probably occurs in CF at least in part because of a combination of voluminous bowel movements and poor nutrition (z), perhaps involving decreased perirectal fat, and may be aggra- vated by secondary manifestations of pulmonary disease such as coughing paroxysms and pulmo,rary overinflation.
meconium ileus & CF
Meconium ileus (10-15% of newborns)
Meconium blocks the ileum
“Meconium ileus is a bowel obstruction that occurs when the meconium in your child’s intestine is even thicker and stickier than normal meconium, creating a blockage in a part of the small intestine called the ileum. Most infants with meconium ileus have a disease called cystic fibrosis.”
CF pulmonary clinical manifestations
80-90% (?) (80-90 experience these SSx (??))
Chronic cough
Purulent sputum (pus)
Hypoxia
Cyanosis
Respiratory failure
CF and barrel chest
As an individual works harder to breathe, they may develop barrel chest
CF and kyphosis
People with CF also report more back and spine pain. These changes to the posture and spine, combined with weakened bones, can result in kyphosis.
CF and pectus carinatum
probably d/t abnormal breathing pattern & mm/bone mechanics
CF and clubbing (digital clubbing)
Clubbing may result from chronic low blood-oxygen levels. This can be seen with cystic fibrosis, congenital cyanotic heart disease, and several other diseases. The tips of the fingers enlarge and the nails become extremely curved from front to back.
why low oxygen lead to digital clubbing?
You make more VEGF when your tissue can’t get enough oxygen, and as a result, you grow more blood vessels, which may change the appearance of your fingers. Clubbed nails occur when your fingertips bulge and the nails curve down and get shiny.
VEFG
Vascular Endothelial Growth Factor (VEGF)
CF – why chronic pulmonary infeciton
Pathogens become trapped in these abnormal lung secretions, leading to chronic infection and persistent inflammation.
CF genitourinary SSx (reproductive system)
Infertility is universal in men and common in women
–> fallopian tubes mucociliary action is comprimised (d/t same reason – thickened mucus secretions w/ excess NaCl)
–> in males, the ductus deferens simple doesn’t even develop
CF and ductus deferens (vas deferens)
Congenital bilateral absence of vas deferens (CBAVD) affects about 1/1,000 males.
It is found in 98% of males with cystic fibrosis and accounts for 6-8% of cases of obstructive azoospermia.
In about 50% of cases, the CBAVD is associated with the absence of the seminal vesicles.
why is it called cystic fibrosis
The name “cystic fibrosis” refers to the characteristic fibrosis and cysts that form within the pancreas.
CF – musculoskeletal clinical manifestaitons
Muscle pain
Decreased bone density
why CF mm pain?
Although the musculoskeletal aspects of CF are less commonly studied, fractures (predominantly spinal), muscle injuries, and joint pain are more commonly seen in the CF population compared to the general public due to their lower bone mineral density, dysfunctional skeletal muscle, and elevated levels of pro-inflammatory cytokines.
CF – endocrine clinical manifestations
Cystic Fibrosis-Related Diabetes (mix of Type 1 and 2)
why CF –> diabetes?
CF can cause scarring of the pancreas because of thick, sticky mucus. This prevents the pancreas from producing normal amounts of insulin so people with CF can have insulin insufficiency and sometimes even become completely deficient.
CF Dx
Genetic testing
Clinical presentation – failure to thrive, respiratory compromise
Sweat test
Pancreatic enzyme tests
Pulmonary function test
CF Tx
Variable depends on the systems involved
Antibiotics
Medications
Chest PT
Adequate nutrition
Supplementation
Lung transplant
which groups live longer with CF?
wealthier – higher socioeconomic class
= better Tx
CF Px
Improving
Median survival rate – 37 years old
Bronchogenic cyst
A rare condition characterized by the formation of a cyst in the middle of the chest, usually behind the manubrium
how are bronchogenic cysts usually found?
Usually found incidentally
(i.e. not generally symptomatic
bronchogenic cyst SSx (?)
Often asymptomatic,
but symptoms may include chest pain,
cough,
shortness of breath
EXTRALOBAR SEQUESTRATION
Extralobar sequestration is a mass of lung tissue that is not connected to the bronchial tree and is located outside the visceral pleura. An abnormal artery, usually arising from the aorta, supplies the sequestered tissue.
extralobar sequestration is considered ..
Considered congenital
extra lobar sequestration – functional tissue ?
Lung sequestration is a rare congenital anomaly characterized by non-functional lung tissue that lacks normal bronchial communication and receives blood supply from an aberrant systemic artery.
Extralobar sequestration (ELS) is less common and usually found in the lower thoracic or upper abdominal regions.
intralobar sequestration
Intralobar sequestration is a mass of lung tissue within the visceral pleura, isolated from the tracheobronchial tree and supplied by a systemic artery.
intralobar sequestration –> is it more or less common than extralobar sequestration?
More common
is intralobar sequestration congenital?
For many years it was thought to be congenital, but is now thought to be acquired.
how is intralobar sequestration acquired?
“The majority of intralobar sequestrations are probably acquired lesions. Patients usually present before the age of 20 years with recurrent infection. At pathologic examination, intralobar sequestration is characterized by inflammation and fibrosis.”
Chest trauma (?)
Common cold (rhinovirus)
Aka – infectious rhinitis
is there fever with common cold ?
usually afebrile
which part of the respiratory tract is affected during common cold?
the upper respiratory tract.
describe the common cold
An acute, usually afebrile, self-limiting viral infection of the upper respiratory tract.
common cold etiology
Rhinovirus (50%)
other viruses that can cause common cold (less common)
Coronavirus
Adenovirus
Parainfluenza virus
Other viruses
parainfluenza define
a disease caused by any of a group of viruses which resemble the influenza viruses.
adenovirus
any of a group of DNA viruses first discovered in ADENOID tissue, most of which cause respiratory diseases.
recall adenoids
Adenoids are a patch of tissue that is high up in the throat, just behind the nose. They, along with the tonsils, are part of the lymphatic system.
common cold clinical manifestations
“Scratchy” or sore throat (pharyngitis)
Sneezing
Rhinorrhea
Nasal obstruction
Cough
Sickness behaviour
“Sickness behaviour” define
Definition. Sickness behavior is a coordinated set of adaptive behavioral changes that occur in physically ill animals and humans during the course of infection. These behaviors include lethargy, depressed mood, reduced social exploration, loss of appetite, sleepiness, hyperalgesia, and, at times, confusion.
hyperalgesia etymoloy
hyper
“algein” = “feel pain”
INFLUENZA
“The flu”
Influenza is a viral respiratory infection causing fever, coryza, cough, headache, and malaise.
coryza define
acute inflammation of the mucous membrane of the nose, with discharge of mucus; a head cold.
from greek:
koruza = nasal mucus
coryza other note about etymology
Greek kóryza, meaning “catarrh.” Catarrh is an inflammation of a mucous membrane and an excessive secretion of mucus. Coryza is a specific kind of catarrh that happens in the nose or nasal cavities.
coryza (google):
“catarrhal inflammation of the mucous membrane in the nose, caused especially by a cold or by hay fever.”
influenza vs mortality
Mortality is possible during epidemics, particularly among high-risk patients.
–> E.g. very old patients, or patients that are ill / immunocomprimised
influenza etiology
Influenza virus most often
Can be other viruses as well (like common cold)
influenza virus – risk factors
Children
Elderly
Chronic disease
Pregnancy
Other disorders
influenza – clinical manifestaitons
Common cold
Conjunctivitis (pink eye)
Chills
Fever
Prostration
Cough
Aches and pain
Headache
Coryza
Scratchy throat (pharyngitis)
Nausea
Vomiting
Abdominal pain
why conjunctivitis during flu?
Some strains of the flu may cause conjunctivitis. However, it’s not nearly as common a cause as adenovirus, Marioneaux says
chills & flu
They are most often associated with a fever. Chills are caused by rapid muscle contraction and relaxation. They are the body’s way of producing heat when it feels cold. Chills often predict the coming of a fever or an increase in the body’s core temperature.
Once the higher temperature is set, your body begins working to increase its temperature. You will feel cold because you are now at a lower temperature than your brain thinks you should be so your body will begin to shiver to generate heat and raise your temperature. This is the chills.
influenza and nausea/vomiting (GI tract SSx)
??
influenza – medical management
Diagnosis - clinical evaluation
Treatment – symptomatic, antivirals
Prognosis - good
____
____
From last slide – why nausea/vomiting?
“These symptoms come from inflammation in your stomach and intestines. (Although the name, gastroenteritis, refers to your stomach and small intestine, inflammation can spread to your large intestine, too). Inflammation is your immune system activating to fight the virus.”
who are the people that die usually from the flu?
very old
already ill
immunocompromised
influenza – COMPLICATIONS
Pneumonia
Encephalitis
Guillain-Barré Syndrome
Renal disease
___
___
Influenza viruses initially infect the upper airways but can directly extend to the lower airways in severe cases, resulting in a viral pneumonia with significant morbidity and mortality.
influenza and encephalitis (compication)
The exact mechanisms by which the virus triggers encephalitis are not fully understood, but it is thought to be related to the immune response and inflammation.
influenza and the pneumonia
Once the immune system is compromised, or while it is fighting a virus, patients have a higher risk of developing more severe infections, such as pneumonia.
most common complication of the flu
Pneumonia is one of the most common complications that can occur as a result of the flu.
The condition can become very serious and even life-threatening (esp old/immunocompromised)
Guillan Barre Syndrome
a disorder of the peripheral nerves, often preceded by a virus infection, usually beginning in the lower limbs and resulting in abnormal sensation and muscle weakness or paralysis.
how long does influenza survive on surfaces?
Influenza A virus can survive for 24 to 48 hours on hard, nonporous surfaces such as stainless steel and plastic, and for approximately 8 to 12 hours on cloth, paper and tissues 20.
how long does rhinovirus survive on surfaces?
“Cold viruses can survive on indoor surfaces for up to seven days, but are infectious only for about 24 hours.”
what is renal disease a complication of the flu?
Although the pathogenesis underlying renal injuries due to influenza A virus has not been delineated, some hypotheses have been advanced, including ATN due to renal hypoperfusion or rhabdomyolysis, glomerular microthrombosis due to DIC, direct viral injury to the kidney, and an altered immune system with systemic …
